Dyspnea

Hemoptysis

Definition :

It means coughing-up blood. It is useful to determine whether the material that is coughed-up contains large volumes of liquid blood, which indicate brisk bleeding, or whether it contains smaller quantities of dark or clotted blood, which would indicate slow bleeding from low-pressure vessel or subsiding bleeding.

Brisk bleeding: is commonly associated with specific focal ulceration of the bronchus, such as” (bronchogenic carcinoma, foreign body, or bronchiectasis).

Slow bleeding: Strongly suggest venous bleeding and is more likely to be the result of increased pulmonary vascular resistance, with secondary increase in flow through the bronchial venous system such as may occur as a result of mitral stenosis or bronchiectasis.

It is also helpful to notice whether the expectorated blood is admixed with sputum or pus. This is valuable, as the site of origin of the bleeding could be determined. Intimate admixture of blood and pus are signs pointing to a deep-seated site of pulmonary suppuration such as pyogenic lung abscess.

Pink, frothy sputum is frequently associated with acute pulmonary edema.

Hemoptysis may be due to pulmonary tuberculosis, pneumonia, bronchiactasis, bronchogenic carcinoma, primary pulmonary hemosiderosis and necrotic arterial lesions due to periarteritis nodosa and lupus erythematosus.

** Three conditions must never be overlooked as causes of hemoptysis:

(1) Mitral stenosis:

-         It is frequently induced by physical exercise, sexual intercourse, or marked excitement.

-         It may be the first symptom, and may occur during pregnancy.

-         The blood comes from a break in the pulmonary veins, which rupture under high pressure.

-         Apoplexy: occurs in 10% of cases with reversible pulmonary hypertension due to rupture of broncho-pulmonary venous varicosities. This type tend to subside as the vein adapt to high pressure and as pulmonary arteriolar disease develops.

(2) Pulmonary infarction:

-         Frank hemoptysis occurs in the minority of instances.

-         When hemoptysis occurs in a patient with heart failure, pulmonary infarction is likely.

-         The bloody sputum usually appears from a few hours to a day after the embolus.

(3) Eisenmenger physiology:

          Patients with severe pulmonary hypertension associated with atrial septal defect, or patent ductus arteriosus, may have hemoptysis, secondary to rupture of pulmonary capillaries.

** Four additional rare causes of hemoptysis:

1-     Rupture of a pulmonary arteriovenous fistula.

2-     Rupture of aortic aneurysm into the trachea or bronchus.

3-     Pulmonary hemosiderosis.

4-     Bronchial ulceration due to foreign body.

Cyanosis

Definition :

Bluish coloration of skin and mucous membrane.

Cyanosis cannot occur when the hemoglobin is less than 33 percent of normal since reduced hemoglobin cannot be produced in an amount sufficient to cause the bluish color (Five grams of reduced hemoglobin is needed for cyanosis to occur). When the hemoglobin is normal, about one-third of it must be in the reduced form for the bluish color to appear.

Cyanosis may be detected by the patient or by the patient’s family. However many patients are cyanotic and are unaware of it.

When the possibility of congenital heart disease exists the patient is usually a child or adult.

* Clinical significance of history of cyanosis:

1-      If cyanosis is present only during the neonatal period the possibility of an atrial septal defect with temporarily reversed flow (right-to-left shunt) during the neonatal period is suggested.

·         However, the most common causes of neonatal cyanosis are, atelectasis, pneumonia, or even occasionally from cerebral damage.

2-      Persistent and severe neonatal cyanosis suggests a right-to-left shunt often with marked impairment of pulmonary blood flow:

·         Tetralogy of Fallot with pulmonary atresia.

·         The hypoplastic left ventricular syndrome.

·         Tricuspid atresia.

·         Sometimes transposition of great vessels.

3-      Cyanosis that develops after a few years of life suggests a less severe form of tetralogy of Fallot, in about of 75% or more. Cyanosis often appeas following delayed closure of a patent ductus arteriosus, or when child begins to walk.

4-      Cyanosis occuring later in life of childhood is suggestive of Eisenmenger complex (N.B. Occasionally cyanosis may be present early in life with this disorder, but not common).

5-      Patients with trialogy of Fallot (atrial septal defect and pulmonary stenosis), usually describe history of cyanosis, late in (childhood or adolescence).

6-      A history of squatting with severe cyanosis (Hypoxic spells), are most suggestive of:

·         Tetralogy of Fallot.

·         Trialogy of Fallot.

Or        · tricuspid atresia.

N.B.

a.       Squatting seldom occur in Eisenmenger syndrome.

b.      The squatting usually occur to relieve dyspnea and spells of unconsciousness, with severe cyanosis.

7-      In adults cyanosis and digital clubbing may be caused by right-to-left- shunting. Such shunting is most often the result of the Eisenmenger syndrome and occasionally of tetralogy or trialogy of fallot or Ebstein’s anomally, or partial transposition of the great vessels.

8-      Adult cyanosis also may result from chronic or acute pulmonary disease.

a)    Chronic disease as:

·         Chronic obstructive lung disease.

·         Hypoventillation syndromes.

·         Pulmonary infiltrative diseases as Hamman-Rich syndrome, sarcoidosis, metastatic cancer or severe bronchiectasis.

b)   Acute disease:

· Pneumonia.

· Pulmonary embolism.

·· But the patient seldom complains of cyanosis in these problems.

9-      Cyanosis due to stagnant hypoxia:

a)    Distal parts of extremities usually cyanosed in advanced heart failure.

b)   Cyanotic hands may be due to local stagnation called (Acrocyanosis).

10-  Rarely, cyanosis is caused by chronic or acute methemoglobinemia, or apparent cyanosis by argyria, which causes a bluish-gray discoloration of the nail beds and mucosae associated with prolonged use of nose drops containing silver.

11-  Acute cyanosis in patients with chronic heart failure, should suggest the possibility of associated pulmonary embolism.

            When cyanosis is definitely noted, it is necessary to determine if it is central or peripheral.

12-  Very specific physiologic implication can be made when differential cyanosis is found:

 (1) Cyanosis of the fingers greater than that of the toes suggests complete transposition of great vessels with either a preductal coarctation or complete interruption of aortic arch and pulmonary hypertension with reversed shunt through a patent ductus arteriosus delivering oxygenated blood to the lower extremities.

   N.B.   If the left arm is slightly less cyanotic than right arm, the coarctation of aorta is suggested. On the other hand if both arms are intensely cyanosed, complete aortic interruption is suggested.

(2) Cyanosis and clubbing of the toes associated with pink finger nails of right hand and minimal cyanosis and clubbing of left hand are suggestive of pulmonary hypertension with reversed shunt through patent ductus arteriosus with normally related great vessels.

(3) If the right hand is pink and the left hand and lower extremities are intensely cyanosed, this suggest, that the right subclavian artery arises proximal to aortic obstruction, plus patent ductus arteriosus and pulmonary hypertension.

On the other hand, if the right subclavian artery originates anomalously from the descending aorta, then both hands are cyanotic.

                                    Syncope

Definition:

It is a complete and transient loss of consciousness due to inadequate cerebral blood flow. The physician should bear in mind that the commonest variety of syncope (vasodepressor syncope) is not caused by organic heart disease, or by organic disease of the circulation.

Clinical significance:

1-      Syncope that occurs in a threatening situation, such as venipuncture suggests simple vasodepressor syncope.

2-      A history of syncope since childhood when associated with organic heart disease, may suggest the possibility of congenital aortic stenosis.

3-      Syncopal spells occur with tetralogy of Fallot, in such a case, it is associated with dyspnea & cyanosis.

4-      In children syncope rarely occurs in primary pulmonary hypertension.

5-      Syncope seldom occurs in congenital complete atrioventricular (AV) block since the heart rate is usually greater than 55/min. and the cardiac output reasonably well maintained.

6-      In older patients a history of syncope may be related to one of several variaties of organic heart diseases:

-          If the patient is thought to have mitral stenosis, a history of syncope may be very significant, it seldom occurs with uncomplicated mitral stenosis. Such a history suggests the possibility of a left atrial myxoma, or ball-valve thrombus with intermittent obstruction to left ventricular filling.

7-      Syncope in patient with ESM & LVH :

a.       If typically occurs with exercise suggests aortic stelosis.

b.      If it occurs after stopping exercise suggests HOCM.

8-      A history of syncope in patient with angina should raise the possibility of aortic valvular stenosis.

9-      Syncope rarely occurs in patient with ischemic heart  disease, although occasionally it occurs at the outset of acute myocardial infarction.

10-  Syncope may occur in patients who have paroxysmal tachycardia.

11-  Syncope may be the initial symptom of massive pulmonary embolism.

12-  Syncope that occurs while shaving or while suddenly turning the head, especially when wearing a tight collar, suggests carotid sinus syncope.

13-  It is important to have a history of the position in which syncope occurs. The patient may become light-headed or giddy when first arising in the morning and becomes more able to tolerate the upright posture during the day. This history suggests orthostatic hypotension.

14-  Syncope, which is associated with memory defects, temporary hemiparesis, paraplegia, vertigo or tinuitus in an older patient suggests that vertebrobasilar artery insufficiency may be responsible.

                                     Dizziness

It is a heterogeneous symptom, including sensations of vertigo, presyncope, disequilibrium and light-headedness.

It is one of the most complaint in general medical clinics. It is often self-limiting and only rarely relates to life-threatening events, even in elderly patients.

*Common causes include:

-          Peripheral vestibular vertigo, labyrinthitis, Meniere’s disease

-          Central vestibular cerbellovascular tumor.

-          Psychiatric disorders and hyperventilation usually in younger patients.

-          Multicausal.

** Blurring of vision and even petit mal epilepsy may be revered as dizzy spells

                          Giddiness

blurring of vision associated with altered consciousness and swaying sensation.

                                  Vertigo

Literally it means “sense of turning” ® “rotation” either of the one’s body or of the surroundings.

** The history is an easy pointer to vertigo:

-          Patient reports objects in his environment spun around

-          Or his body was turning.

-          Or his head was spinning.       

*        Sometimes he may state that the floor or walls seemed to undulate or sank or rose-up toward him or he was feeling of to and fro or up-and down movement of the body, usually head.

*        The symptoms of vertigo are usually paroxysmal and of short duration but may be chronic following acute attack.

It is accompanied by varying degrees of nausea and vomiting, nystagmus headache and ataxia, as well as by the need to avoid movement of the head.

* Raynaud’s phenomenon:

Definition:

It is a particular sensitivity of the hands and fingers to cold. Upon exposure to cold the fingers exhibit a typical triple response.

·         First ® Pallor and blanching (white) response.

·         Second ® cyanosis (blue) response.

·         Finally ® rubor (red) response.

-          This (white – blue – red) response, at times may be out of sequence.

-          Also the patients usually have finger numbness or pain.

* Cause:

Spasm of the digital arteries causes ischemia of fingers, with pallar first and cyanosis later (due to increased extraction of oxygen from the trapped and non-circulating erythrocytes) and the final stage of redness coincides with reperfusion, following release of the spasm. This is also the phase of finger numbness or pain.

* Clinical significance of Raynand’s phenomenon:

It usually preceds several important disorders:

1-      Connective tissue diseases.

2-      Disorders characterized by arterial compression (such as thoracic outlet and carpal tunnel syndromes).

3-      Vasculitis and atherosclerotic arterial disease.

4-      Various blood disorders (including cryoglobinemia, monoclonal gammopathy).

5-      Some drugs and toxins.

6-      Miscellaneous disorders: such as hypothyrodism, reflex sympathetic dystrophy, primary pulmonary hypertension, Prinzmetal angina, acromegally, Addison’s disease.

Edema

* Definition: It is an increase in the volume of interstitial fluid (i.e., the extravascular portion of the extracellular compartment). The plasma volume may or may not be increased.

** Interstitial fluid volume may increase considerably before it is clinically appreciated.

** Edema is considered a symptom and a sign.

The patient may give a history of unexplained weight gain, enlarging girth and swelling of the legs, tightness of a ring or shoe & puffiness of the face, swollen extremities and persistence of indentation of the skin following pressure.

* It is important to ascertain whether edema of the extremities preceded or followed dyspnea on effort. Edema of cardiac origin is usually preceded by dyspnea, and usually associated with dyspnea except with tricuspid stenosis.

Local factors play a major role in determining the distribution in body fluid. A good illustration of this, is mitrral stenosis: when diastole is shortened to a critical point, pulmonary edema develops, because the right ventricle continues to pump more blood into the lungs than can pass the stenosed mitral valve.

® Patients with pulmonary edema may exhibit this watery, frothy, blood-tinged sputum, associated with profound dyspnea and wheeze.

® Patients with chronic heart failure who has gained weight because sodium and water retension, secondary to altered renal function may detect edema of the ankles and lower legs.

-          Hypoproteinemia such as nephrosis and starvation causes edema, also in the dependent portions of the body. Such edema usually occurs when the total blood protein is below 5 g/100 ml.

* Edema of one leg:

            - Varicose veins.

            - Thrombophlebitis.

            - Lymphedema.

* When there is bilateral leg edema due to heart failure, there may be more in one side than the other, if a local factor is also present.

* Edema may shift from the extremities to the sacral region when the patient is confined to bed.

* Periorbital edema:

It is  common in children than adult.

It is  common due to renal causes, but may occur in heart failure.

** Rare causes of periorbital edema: (superior vena caval obstruction, and trichinosis).

* Ascitis:

-          It is recognized by the patient as an increase in girth or swelling of the abdomen.

-          Ascitis due to heart failure is uncommon to day and usually follows peripheral edema.

-          A local factor such as cirrhosis is also suggested when ascitis associated with heart failure, seem to be out of proportion to peripheral edema.

-          Constrictive pericarditis and endomyocardial fibrosis are rare causes of cardiac causes of ascitis.

* In children:

Hepatomegally and ascitis are the first symptoms & sign of cardiac edema than peripheral edema due to:

(1)    Portal circulation of children is less complaint.

(2)    Systemic circulation in children is more complaint.

* Edema is a symptom and also it is a sign.

On examination it can be elicited as follows:

-          Press on the skin over a bony area with 3 fingers spread apart for 10-30 seconds.

-          Feel and look of at the valleys produced.

* It is of two types.

a)    Slow edema: the pitting remains for more than 60 seconds, and it is usually due to congestion.

b)   Fast edema: the pitting remains less than 40 seconds, and it is usually due to hypoalbuminemia.

* Edema could be elicited only in presacral area if the patient is bedridden.

                                Fatigue

Definition:

It is a sense of weakness, described by the patients variously as exhuastion, tiredness, lack of pep and energy, loss of ambition or interest, low vitality or a feeling of being “all in”. It is often accompanied by a subjective sensation of weakness and a strong desire to rest and sleep.

• There are many causes of fatigue and weakness and therefore these symptoms are not specific for heart diseases.

* When a patient with heart disease is water-logged or when there is pulmonary congestion due to heart disease, the patient is likely to complain of dyspnea.

* Fatigue in a cardiac patient may be due to:

 - Heart failure.

-          The complain of fatigue may occur just before, during or following myocardial infarction.     

-          Fatigue may be the main complaint of patient with myocardial ischemia.

-          Cardiovascular drugs:

·         Diuretics.

·         B-Blockers.

·         Antihypertensives that cause postural hypotension. 

Hoarseness of voice

-          It is usually unrelated to cardiovascular disease.

-          It may occur in patients with:

1. Aortic aneurysm that involves the recurrent laryngeal nerve.
2. Mitral stenosis, occasionally may cause hoarseness (Ortner’s syndrome), but this symptoms is very rarely today, because mitral stenosis is usually earily corrected either by percutaneous ballon mitral valvuloplasty or surgury.
3. Pericardial effusion, may be related to myxoedema which may produce a coarse, low-pitched voice.
4. Sometimes in patients with larger ductus arteriosus.

               Intermittent claudication

Intermittent pain of the skeletal muscles due to temporary ischemia that is usually (but not always) caused by atherosclerosis of the artery or arteries that supply a particular anatomic part of the body.

·         It is produced by effort and relieved  by discontinuing the effort.

·         It is never related to emotional stress.

-          The arterial obstruction is located proximal to the site of claudication.

*        Leriche syndrome; Intermittent claudication of the thighs and buttocks, is caused by obstructive disease of the iliac arteries or the terminal aorta.

*        Intermittent claudication of the masseter muscles suggests takayasu disease.

*        The “blue toe” syndrome is caused by cholesterol emboli of the toes from proximal atherosclerotic lesions of the arteries proximal to the feet.

*        Acute and persistent pain in the calf, thigh or other muscle group suggests an embolus to the artery or acute thrombosis of an obstructive lesion.                 

Cardiac Cachexia

Patients with longstanding heart failure experience loss of weight and emaciation.

The heart failure usually of more than 5 years duration and the NYHA class is usually of grade III to IV.

Mechanisms:

1.      Poor eating and digestion: due to gastrointestinal congestion and anorexia.

2.      Poor absorption also due to gastrointestinal congestion.

3.      Deficit metabolism: liver congestion.

** Tumor necrosis factors, play an important role in initiation and prolonged cardiac cachexia . All of these factors lead to caloric, protein and vitamin malnutrition.

                             Fever and chills

(1)            Fever in patients with infective endocarditis usually is associated with chills .

(2)            Rheumatic fever is associated with elevation of temperature but chills occur only after initiation of aspirin therapy.

(3)            Acute myocardial infarction may be associated with low grade fever.

(4)            Pulmonary embolism with pulmonary infarction may be associated with low grade fever.

          **Family History**

At times the family history is very helpful in suggesting a specific etiologic variety of heart diseases.

a)      Hypertension: The majority of patients with essential hypertension have a family history of hypertension, and the absence of such family history may suggest that one should look more actively for secondary hypertension.

b)      Coronary artery disease: Although coronary artery disease is considered to be of uncertain genetic background most physicians, have the impression that it does tend to be familial. So a family history of diabetes, hyperglycemia., or hypercholesterolemia suggests the possibility of coronary artery disease.

c)      Congenital heart diseases: Several congenital heart diseases are known to occur in families: for example:

-          Atrial septal defect.

-          Diffuse subvalvular aortic stenosis. 

-          Supravalvular aortic and pulmonic stenosis.

-          Ventricular septal defect.

-          Pulmonary branch stenosis.

d)      Rheumatic heart disease:

Although most patients with rheumatic heart disease do not have a family history of  rheumatic heart disease, it is considered by some to have several associations. Occasionally one sees a family with a number of members who have rheumatic heart disease.

e)      Some connective tissue disease which have a heritable base like:

-          Homocystinurea 

-          Osteogenesis imperfecta

-          Ehlers-Danlos syndrome

-          Marfan’s syndrome 

f)       Osler-Weber-Rendu disease or hereditary telangiectasia is known to be a familial disease, and approximately 15% of such cases have pulmonary arteriovenous fistulas.

Significance of The History As A Pointer To The Diagnosis

Cardiomyopathy

a)      Hypertrophic cardiomyopathy:

-        Family history of sudden death

-        Younger or middle-aged patient.

-        Anginal pain or syncope, after but not during exertion.

-        Recurrent attacks of palpitation.

-        Worsening of the symptoms on digitalis therapy.

b)      Dilated cardiomyopathy:

1.      Influenza-like illness, followed by symptoms & signs of heart failures.

2.      Short-term, history of dyspnea.

3.      History of drug-intake that is known to cause myocardial depression.

4.      Peripartum symptoms & signs of HF suggestive of Peripartum CM.

c)      Restrictive Cardiomyopathy

1.      History of frequent blood transfusion, skin color changes, recurrent arthritis, impotence with diabetes mellitus may suggest hemochromatosis.

2.      Recurrent attacks of bronchial asthma in young patient with history of embolic manifestations may suggest esinophilic restrictive cardiomyopathy.

3.      History of attacks of postural hypotension, with skin bleeding and scratching may suggest amyloidosis.

4.      Recurrent eye symptoms (uveitis), history of kidney stone and renal problems and recurrent attacks of syncope may suggest sarcoidosis.

Hypertension:

1.      Age <35 years, search for secondary hypertension.

2.      Flank trauma or flank pain may point to renal ischemia.

3.      Medication history as contraceptive pills and weight loss preparation and herb teas used for weight loss.

4.      History review other system:

a.             Polyuria, polydepsia or nocturia may suggest, renal or endocrinal causes especially in children.

b.            Weight gain, ecchymoses, edema, new acne, change in libido or behaviors or change in the menstrual pattern in female may point to cushing syndrome.

c.             Cold legs & claudication may suggest aortic coarctation.

d.            Tetany, nocturnal polyurea, episodic or continual weakness may point to aldosteronism.

e.             Polyurea, renal calculi, prolonged history of constipation, gastric ulcer may suggest hyperparathyrodism.

f.             Oligomenorrhea and hirsutism may accompany cushing, thyroid or adrenogenital syndrome.

g.            Headache, diaphoresis, palpitation, postural hypotension, flushing and heat intolerance may suggest pheochromocytoma.

5.      Family History:

a.             Positive family history ® may point to essential hypertension.

b.            Lack of family history especially in younger patient turns the attention towards secondary hypertension.

Cardiopulmonary disease

a)      Chronic obstructive lung Disease (COLD)

-        Heavy smoking history.

-        History of seasonal asthmatic attacks.

-        History of repeated asthmatic attacks since childhood, helped with bronchodilator.

-        The patient may give a history of easier breathing on leaning forward.

-        Chronic cough and wheezing.

-        Occupational history e.g (coal-miner workers).

b)     Pulmonary emboli with core-pulmonale:

-        History of long sitting after surgery.

-        Pregnancy & delivery.

-        Trauma.

-        Phlebitis and oral contraceptives.

-        History of deep venous thrombosis.

c)      Primary pulmonary hypertension:

-        Raynaud’s phenomenon.

-        Family history of primary pulmonary hypertension (6%).

Rheumatic Heart Disease:

History suggestive of Rheumatic fever:

1.      Polyarthritis:

-        Fleeting or migrating arthritis.

-        Redness, hotness & swelling.

-        Associated with complete loss of mobility.

-        Self-limited.

-        Shows a dramatic response to Aspirin therapy.

-        No permanent deformity.

2.      History of fever with murmur.

3.      long-acting of penicillin taking history.

4.      Family history.

5.      History of murmur during school examination, insurance or military service.

Congenital Heart Disease:

1.      Family history of a congenital heart disease.

2.      History Regarding pregnancy:

-        Drug history during pregnancy.

-        Rubella infection; may lead to:

§  Atrial septal defect

§  Ventricular septal defect.

§  Fallot’s tetralogy.

§  Pulmonary branch stenosis.

§  Patent ductus arteriosus.

-        Viral illness may lead to neonatal myocarditis.

-        Diabetic pregnant woman: the diabetic may be associated with (transposition and Fallot’s tetralogy.)

3.      Cyanosis:

a.       Time of appearance:

-        Since birth: (transposition, Fallot’s tetralogy.)

-        Childhood: (Eisenmenger’s syndrome or Ebstein’s anomaly)

-        Adult with pulmonary hypertension may suggest ASD.

b.      During infancy associated with crying or feeding, may suggest Fallot’s tetralogy.

c.       Squatting: may suggest.

-        Fallot’s tetralogy.

-        Pulmonary atresia.

-        Eisenmenger syndrome.

d.      Differential cyanosis: suggests ductus with  Eisenmenger syndrome.

e.       Stroke in cyanotic patient, considers cerebral abscess e.g. in  Fallot’s tetralogy.

f.       Cyanosis & hemoptysis may suggest congenital arteriovenous fistula.

4.      History of headache, leg fatigue, intermittent claudication, cold legs and recurrent epistaxis in childhood and young adult, may suggest coarctation

5.      Child with mental retardation.

6.      History of frequent pneumonia during infancy and childhood, may suggest increased lung blood flow (left to right shunt).

Ischemic heart disease:

1.      Careful history, may point to ischemic nature of chest pains. The key features include:

-        Character and quality of the pain.

-        Location and radiation.

-        Precipitating, exacerbating and relieving factors.

-        Duration of pain.

-        Associated symptoms.

2.      The presence of any of the Five major risks with the chest pain:

-        Hypertension.

-        Hyperlipidemia (dyslipidemia).

-        Diabetes mellitus.

-        Smoking.

-        Family history of premature coronary artery disease.

3.      Marked postprandial somnolence may suggest severe hypertriglyceridemia.

History suggestive of pericardial diseases:

1.      Medical history:

·         Uremia

·         Metastatic carcinoma, lymphoma, leukemia.

·         Connective tissue disorders as rheumatoid arthritis.

·         History of skin rash after direct sun exposure (systemic lupus)

·         History of tuberculosis.

·         Recent viremia.

2.      Medicinal history:

·         Chest radiation (recurrent).

·         Chemotherapy.

·         Isoniazid, or minoxidil.

3.      Patient symptoms:

·         Chest pain: increased on lying flat and relieved on leaning forward.

·         Epigastric pain 1-3 days before chest pain.

·         Abdominal swelling before lower limb edema and just before beginning of dyspnea or simultaneous with dyspnea on exertion.

History of recurrent arterial and venous thrombosis, recurrent spontaneous abortion may suggest.

·         Primary antiphospholipid antibody syndrome.

·         Systemic lupus erythematosus.

·         Thrombophelia positive patients(especially women).

History of skin abnormalities on direct exposure to sun light may suggest systemic lupus  erythematosus.

History of embolic manifestation associated with constitutional symptoms and syncopal attacks with postural changes may suggest atrial myxoma.

History of intermittent flushing, recurrent wheezing, diarrhea, may suggest carcinoid heart disease.

General signs

The experienced physician always observes the patient while taking the history of the illness.

For Example:

1-      General appearance: whether the patient appears comfortable or not.

2-      Is there is obesity or emaciation.

3-      Mental status: is judged from consciousness, orientation and ability to interpret questions, and to make appropriate responses.

4-      Facial appearance and expression, speech, cyanosis, excessive perspiration, dyspnea, orthopena, tremor. Thus the physical examination cannot be completely separated from the process of history-taking. The cardiac examination of course, is not ordinary carried-out separately from the general examination.

1- Voice:

-          The quality of the patient voice should be observed.

-          A very husky voice of low pitch may be significant specially if there has been a recent change.

-          In the absence of acute laryngitis such as voice may be associated with myxedema.

-          Also may suggest the possibility of recurrent laryngeal nerve palsy due to aortic aneurysm or tumor such as carcinoma or papilloma of thorax.

-          Aneursmal dilatation of left atrium may press on the left recurrent laryngeal nerve (Ortner’s syndrome).

2- Body Appearance :

-          If the patient is quite ill the position that he assumes for greatest comfort may be a good pointer for cardiac disease.

1-      The patient with Fallot’s tetralogy, may squat for relief of dyspnea, but this posture is rare in adult patient, particularly in public area.

2-      Dyspnic patient due to, orthostatic hypotension, left atrial myxoma or left atrial ball-valve thrombus may obtain relief by lying down.

3-      Patient with pericarditis may be most comfortable sitting up and leaning forward.

4.      Levine’s sign: clenched fist over the chest of a patient with angina pectoris

5.      Waddling gait, lumber lordosis, and calf pseudohypertrophy of Duchenne muscular dystrophy may be associated with:

·         Hypertrophic cardiomyopathy .

·         Pseudoinfarction pattern in ECG.

6.      Hammer toes and pes cavus of Friedreich ataxia, may be associated with:

·         Hypertrophic cardiomyopathy.

·         Sick sinus syndrome.

·         Angina pectoris.

7.      Straight back of ankylosing spondylitis associated with aortic regurgitation and complete heart block.

8.      Struggling, anguished and frightened look of patient with pulmonary edema, sitting upright in bed, tachypneic and diaphoretic.

9.      Mesomorphic, overweight, balding hairy, and tense middle-aged patient prone to coronary artery disease.

10.  Anasarca of congestive heart failure.

11.  Tall stature, long extremities and sparse. This may be associated with mitral valve prolapse and aortic root dilation.

12.  Tall stature and thick extremities of acromegally. It may be associated with hypertension, cardiomyopathy and conduction defects.

13.  Short stature, webbed neck, low hairline, small chin, wide-set nipples and sexual infantilism of Turner syndrome. It may be associated with valvular pulmonary stenosis, and aortic coarctation.

14.  Dwarfism and polydactyly of Ellis-Van Creveld syndrome. It is usually associated with atrial  saptel defect and single atrium.

Mouth

1.      Enlarged tongue:

-        Amyloidosis.

-        Glycogen storage disease.

-        Hurler’s syndrome.

-        Down’s syndrome.

-        Hypothyroidism.

2.      Orange, large lobulated tonsils ® occurs in Tangier disease (type of hyperlipidemia.)

3.      Petechiae on buccal mucosa, occurs in bacterial endocarditis.

4.      A high arched palate can be observed in Marfan’s syndrome.

5.      Cleft palate is associated with a high incidence of different types of congenital heart disease:

-        Atrial septal defect.

-        Patent ductus arteriosus .

-        Dextrocardia.

-        Coarctation of aorta.

Ears

1.      Deafness is common in Hurler’s syndrome, Turner’s syndrome, klippel-Feil syndrome, osteogenesis imperfecta, rubella syndrome, familial pulmonary stenosis, familial mitral regurgitation with skeletal anomalies.

2.      The associatation of cataracts, deafness, nystagmus, and peripheral pulmonary branch stenosis or patent ductus arteriosus.

3.      Deafness and a prolonged Q-T interval on ECG may be familial.

4.      Bounce (pulsation) earlobe, with venous pulsation is an excellent sign of severe heart failure, and this sign can differentiate between venous and arterial pulsation. Where the arterial pulsation infrequently moves earlobe.

5.      An increased incidence of a diagonal earlobe crease is curious observation in patients with coronary artery disease.

Obesity

Obesity may be central or peripheral. In fact its location correlates strongly with its effect on health.

(a) Central obesity:

·  Involves primarily the trunk and it is characterized by:

1-      A bihumeral diameter is greater than bitrochanteric diameter.

2-      Subcutaneous fat has a descending distribution and is concentrated primerily in the upper half of the body (neck, cheeks, shloulder, chest and upper abdomen).

(b) Peripheral obesity:

            Involves primerily the extremities, and it is characterized by:

-          A bitrochanteric diameter is greater than the bihumeral diameter.

-          Subcutaneous fat has an ascending distribution and is concentrated primerily in the lower half of the body (lower abdomen, pelvic girdle, buttocks, and thighs).

** Central obesity is more common in men, while peripheral obesity is more common in women.

** Central obesity carries a much worse prognosis because of its higher association with hypertension,diabetes, atherosclerotic cardiovascular diseases, dyslipidemia and lower extremity venous stasis diseases.

* Body Mass Index (BMI):

-          It is the federal government’s standard for body weight.

-          It is calculated as a ratio between weight and height and provides a better measurement of body fat than the traditional height and weight charts.

® According to old standards:

(1)    Men were considered overweight if they had BMI ³ 27.3.

(2)    Women were considered overweight if they had BMI ³ 27.8.

®Revised guideline: Any one with BMI ³ 25 is considered abnormal

BMI and risk adjustment.

* Exceptions to BMI:

Although the BMI is a better predictor of disease risk than body weight alone, certain people should not use the BMI to determine their risk. For example:

(a)     competitive athletes and body builders. Because their larger muscle mass, their mass index is high.

(b)      pregnant and lactating women.

(c)      growing children and frial, sedentary elderly people.

Pectus Excavatum

It is also known as funnel breast. It has a number of associations of interest to cardiologists.

It may be considered severe when the distence between posterior sternum and the vertebral column is less than 5 cm, and moderate when the distance is (5-7) cm.

N.B. The normal average for men is 10.5 cm and for women is 9.0 cm.

* Clinical Significance:

The following associations should be born in mind on examining a patient with funnel-breast deformity:

1- Marfan’s syndrome:

Patient with funnel-breast deformity should be examined for other features of Marfan’s syndrome including ectopia lentis, aortic regurgitation,  kyphoscoliosis, lax ligaments and family history of the disease.

2- Homocystinuria:

• Rare than Marfan’s syndrome.
• The cardiovascular abnormalities usually include thrombi involving coronaries and other systemic arteries or systemic veins.

3- Ehler-Danlos syndrome:

Presents in about of 2% of patients with pectus excavatum.

4- Mucopolysaccaridosis:

As the Hunter-Hurler syndromes.

5- Mitral valve prolapse:

Approximately 25% of patients with mitral valve prolapse have thoracic deformity including pectus excavatum.

6- Cardiac compression:

Cardiac compression with elevation of systemic and pulmonary venous pressure had been reported even vay rare.

7- Pulmonary disease:

Vital capacity and maximum breathing capacity may be lowered. Core-pulmonal may be developed.

8- Pseudo-heart disease:

The most important association for the cardiologist is that heart disease may be suggested by usual radiologic physical or electrocardiographic findings.

(a) Physical examination reveals:

-          Pulmonary ejection murmur.

-          Expiratory splitting of second sound.

-          An ejection click.

-          The cardiac apex may be displaced to left.

These findings may incorrectely diagnose atrial septal defect, pulmonic stenosis or cardiac enlargement.

(b) The radiogram shows:

-          Displacement of the heart to the left.

-          Absence of right atrial shadow to the right of the spine.

-          The heart may be flattened antero-posteriorly and thus appears enlarged in antero-posterior radiography (Panacke Heart).

(c) The Electrocardiogram:

may show:

-          Negative T-waves in right precordial leads.

-          Deeply negative P wave in (V₁).

-          QRS voltage is small in (V₁) and larger in (V₆).

-          RSR` pattern may occur in V₁.

Shield chest:

It is a broad chest with a great angle between the manubrium and body of the sternum, as well as widely separated nipples.

1. In male: It is commonly associated with pulmonary stenosis and called Noonan or Ulrich’s syndrome.
2. In female: It is associated with neck webbing and short stature, and aortic coarctation. It suggests Turner’s syndrome. 

Cardiovascular Facies

1- Elfen facies: is characterized by

• Short and upturned nose.
• Widely spaced eyes.
• Full checks.
• Wid mouth with patulous lips.

** Deep husky voice, and friendly personality.

** It is often associated with:

·         Hypercalcemia.

• Supravalvular aortic stenosis.
• Mental retardation.

2- Corvisart’s facies: is characterized by:

·         Puffy, purplish and cyanotic & swollen eyelids.

• Shiny eyes.

** It is observed in patients with:

-Aortic regurgitation.

      -Heart failure.

3-Aortic facies:

-          Another face of patients with aortic regurgitation.

·         Its hallmark is a pale and sallow complexion.

4- DeMusset’s facies:

            It is the bobbing motion of the head, synchronous with hear tbeat.

* Significance:

1-      It is first characterized as a typical facies of patient with AR.

2-      It is neither sensitive nor specific.

3-      In fact it is usually observed in hyperkinetic states (large stroke volume).

4-      There is a variant of DeMusset’s sign, seen in severe tricuspid regurgitation but the bobbing of the head, tend, to be more lateral as a result of the regurgitant column of blood rising along the superior vena cava.

5-      It may be observed in patient with massive left pleural effusion.

5- Mitral facies:

It is the face of mitral stenosis. It is characterized by pink and slightly cyanotic cheeks. This form of cyanosis affects primerily the periphery of the body (i.e., acrocyanosis), such as the tip of the nose, cheeks, hand, and feet. It is due to peripheral desaturation caused by low and fixed cardiac output.

When patients with mitral stenosis develop right-sided heart failure and tricuspid regurgitation as a result of long-standing pulmonary hypertension, the overall skin color becomes sallow and often overty icteric. This appearance contrasts quite well with persistently cyanotic cheeks.

6- Facies of lupus erythematosus:

            It is characterized by:

-          A classic malar, butterfly-like-rash, and often involving the bridge of the nose.

7- Other clinical presentations:

(a)             Large occluding pulmonary embolus.

• Calamitous onset of cyanosis of face.
• Cold sweating of forehead.
• Neck vein distension, with prominent A-wave.
• Dyspnic facies.

(b)   Acromegally.

• Excessive growth of facial bones.
• Broad forehead.
• Protruding mandible.

** Associated with:

-          Conduction defects.

-          Cardiomyopathy (due to hypertrophy & fibrosis).

-          Coronary atherosclerosis.

(c)    Hypothyroidism:

-          Dull face with distorted thick skin.

-          Coarse features.

-          Dry hair.

-          Puffy eyelids.

-          Enlarged tongue.

-          Loss of outer 1/3 of eye brows.

** It is associated with:

-          Pericardial effusion.

-          Hypercholesterolemia.

-          Possibly premature ischemic heart disease.

(d)   Butterfly Rashs:

·         Systemic lupus erythematosus is associated with libman-sacks endocarditis.

(e)    Malar flash, without Rash: may be seen in patients with severe mitral stenosis.

(f) Facial dysmorphism:

• Microcephally.
• Triangular face, a broad forehead, a small mouth, facial asymmetry and large posteriorly rotated ears.

® this facies is usually associated with right sided aortic arch.

Eye

Many cardiovascular clues could be suspected from eye examination:

(1)   Exophthalmos:

• Suggests hyperthyroidism.
• Exophthalmos plus systolic pulsation may be seen in severe TR.

(2)   Edematous lids, loss of outer brow hair & sleepy appearance ® hypothyroidism.

(3)   Oculomotor palsy may suggest ® association of complete heart block.

(4)   Ectopia lentis,. Iridodonesis ® Marfan’s syndrome or Homocystinurea.

(5)   Blue sclerae may be found in:

• Mafran’s syndrome.
• Ehler’s-Danlos syndrome.
• Osteogenesis imperfectae: Usually associated with AR & MR.

(6)   Arcus senilis in patients under 40 years ® hyperlipidemia.

(7)   A coloboma (fissure) of iris and choroid is a major sign of the “Cat eye” syndrome is associated with:

-          total anomallous pulmonary venous drainage.

(8)   External ophthalmoplegia, ptosis, myocardial disease and complete heart black ® (kearns-sayre syndrome).

(9)   Cataract:

-          Marfan’s syndrome.

-          Down’s syndrome.

-          Homocystinurea.

** Laurence-Moon-Biedl-Bardet syndrome is associated with:

• Complete transposition of great vessels.
• PDA.
• Valvular pulmonary stenosis.
• Dextrocardia.
• Hypoplastic aorta.

(10)    Xanthelasma: of eye lids signifies hyperlipidemia especially young persons.

(11)    Cushing’s syndrome is usually associated with hypertension.

(12)    Reiter’s syndrome:

• Conjunctivitis associated with aortic regurgitation.
• Conduction defects.
• Myocarditis.
• Pericarditis.

(13)    Bacterial endocarditis:

** may lead to infected emboli.

-          may present with severe ophthalmitis.

-          Subconjunctival hemorrhage.

-          Petechiae.

(14)    Epicanthal folds of mongoloidism may be associated with:

-          Common A-V canal.

-          Ventricular septal defect.

-          Fallot’s tetralogy.

(15)    Jaundice: It is not often seen with cardiac problems.

It may be seen in:

1. High output states due to hepatic cirrhosis and alcoholic hepatitis & may be associated with cardiomyopathy.
2. Cardiac cirrhosis due to advanced and long-standing heart failure.
3. Pulmonary embolism and infarction.
4. Red-cell hemolysis due to prosthetic valves.

N.B.: The serum billirubin level seldom rises above 6 mg/100 ml, even in severe heart failure. If it is above 6 mg/100 ml, additional problem plus heart failure is suspected.

Hands

1-    Clubbing and cyanosis:

            These are typical of congenital heart disease or pulmonary arteriovenous fistula with a right to left shunt.

* Differential cyanosis and clubbing:

            It has a specific physiologic implication:

(a)    Cyanosis of fingers is greater than that of the toes; suggests transposition of great vessels with either a preductal coarctation or complete aortic arch interruption with pulmonary hypertension, and reversed shunt. In this case, if the left arm is less cyanotic than right, coarctation of aorta is suggested. On the other hand, if the cyanosis is intense and symmetrically, aortic arch interruption is suggested.

(b)   Cyanosis and clubbing of the toes, associated pink fingernails of right hand and minimal cyanosis of left hand: this suggests patent ductus arteriosus with normally related vessels and pulmonary hypertension with reversed shunt.

(c)    Cyanosis & clubbing of the toes and left hand while the right hand is not cyonatic, this suggests, the right subclavian artery arises proximal to coarctation of aorta.

* Reversed differential cyanosis:

-          Hands are cyanotic and clubbed, but the feet are normal.

-          This occurs when there is right ventricular origin of both great vessels, with concomitant disorder including VSD, PDA & pulmonary hypertension ® oxygenated blood from LV enters the pulmonary trunk through VSD, or from PDA into descending aorta so the feet are normal. While the oxygen-desatrurated blood from RV enters the ascending aorta and brachiocephalic vessels so the upper extremities are cyanosed and clubbed.

* Unilateral clubbing:

-          Aneurysm of aorta, or innominate/subclavian arteries.

-          Pancost’s tumour and lymphangitis.

2-    Red fingertips, “tuft erythema”: it may signify small or intermittent right to left shunts with only slight reduction in oxygen saturation.

3-    Acute painful clubbing or hypertrophic osteoarthropathy is a manifestation of bronchogenic carcinoma, which may invade or metastasize to the heart and pericardium.

4-    Quinke’s pulsations: it is flushing of nail beds, synchronously with heart beats. It is a sign of high output states as aortic regurgitation.

5-    Splinter hemorrhage; they are:

-          Longitudinal, black and splinter shaped.

-          Usually located in the distal third of the nail.

* They may suggest infective endocarditis.

* Trichinosis

* Commonly result from trauma.

6-    Osler’s nodes:

-          They are reddish purple, raised, tender nodules in the distal pad of fingers or toes.

-          They suggest infective endocarditis

7-    Janeway lesions : small non-tender raised erythematous or hemorrhagic leasions of palms or soles:

-          They may suggest infective endocarditis.

8-    Rheumatoid arthritis:

*        There is ulnar deviation of the fingers, thickening of the middle interphalangeal joints, boxing of the wrists and subcutaneous nodules.

*        There is a high incidence of cardiac involvement includes:

·         Pericarditis.

·         Coronary arteritis.

·         Granulomatous inflammation involving myocardium or base or cusps of aortic and mitral valves causing AR and MR.

9-    Jacoud’s artheritis:

-          Marked ulner deviation at the metacarpophalangeal joints.

-          It is almost always due to repeated attacks of rheumatic fever activity.

* The deformity is due to periarticular fascial and tendon fibrosis, rather than synovitis. So the fingers can be moved freely into correct alignment.

10 –Whipple’s syndrome is charaterized by:

· Polyarthritis.             · Abdominal pain.

· Diarrhea.                               · Pericarditis and endocarditis.

11-Deposition of uric acid crystals ® gouty artheritis may be associated with gouty nodules in myocardium, valves or conducting system.

12-Systemic lupus erythematosus:

Hand lesion:

• Inflammation of proximal interphalangeal or metacarpophalangeal joints.
• Erythema of fingertips.
• Vascular blush over phalanges and sparing the knuckles.
• Brownish red macular rash of the palms. Raynaud’s phenomenon and vitiligo.

Heart lesion:

• Pericarditis.
• Verrucous endocarditis
• Thining and perforation of the valves.
• Conduction defects.

13-Hyperthyroidism:

-          Moist hand.

-          Fine tremors.

-          Sometimes clubbing of fingers.

14-Hypothyroidism:

-          Cold hand.

-          Coarse, puffy skin.

15-Acromegaly:

-          Spadlike hand.

-          Sausage fingers.

16-A variation in the size or number of fingers is an excellent indication of congenital heart disease:

(a) Holt-Oram syndrome:

1. The thumb may have an extra phalanx (fingerized thumb).
2. Distal radial and ulner deformities; causing difficulty in supination and pronation.
3. It is usually associated with secundum atrial septal defect (ASD).

(b) Ellis -Van Creveld syndrome:

• Polydactyly.
• Extra-fingers and sometimes extratoes.
• Hypoplastic finger nails.
• Dwarfism.

* It may be associated with:

• Primum ASD.
• Ventricular septal defect.
• Endocardial cushion defect.
• Single atrium.

(c) Hurler’s syndrome:

-          Broad hand.

-          Stubby fingers.

-          Clinodactyly (curving of the fifth finger radially).

-          Flexion contractures with claw hand.

Reminders

* Clubbing:

* Definition:

Digital clubbing is focal enlargement of the connective tissue in the terminal phalanges of the fingers and toes; it is especially prominent on the dorsal surface of the digit. It is usually painless, although at times patients may complain of an aching discomfort in their fingertips.

* Diagnostic features of digital clubbing:

It depends on whether clubbing is present alone or in association with periostosis.

(a) Clubbing without periostosis, has three main features:

(1) Loss of lovibond’s angle: It is the angle between the base of nail and its surrounding skin.

-          In normal person it is less than 180°.

-          In a person with clubbing the angle may be obliterated or it is greater than 180°.

*        The loss of the angle can be visualized by resting a pencil over the nail. In normal, there is a clear window below the pencil and the nail. In case of clubbing there is no clear window and the pencil rests fully over the nail.

* Schamroth’s sign:

-          It is another bedside maneuver that can confirm the loss of the subungual angle.

-          It consists of the disappearance of the diamond-shaped window normally present when the terminal phalanges of paired digits are juxtaposed.

(2) Floating nails (ballottability of the nail bed):

It is increased sponginess of the soft tissue at the base of the nail. The nailplate acquires a peculiar “springy” feeling. When the skin just proximal to the nail is compressed, the nail sinks deep towards the bone; when released, it springs backward and outward (floating finger nail base).

(3) Abnormal phalangeal depth ratio:

It is consists of a greater depth of the finger-tip when measured at the cuticle (distal phalangeal depth [DPD]) Vs. the interphalangeal joint (interphalangeal depth [IPD]). The DPD/IPD ratio of normal people is, on average, 0.895. Patients with clubbing have a DPD/IPD ratio equal to or greater than 1.0 (i.e., in excess of the normal by approximately 2.5 standard deviations). The DPD/IPD ratio is an excellent marker for clubbing.

* Types of digital clubbing:

1. Parrot’s beak: ® the swelling is primarily localted to the proximal portion of the distal digit.
2. Watchglass: ® the swelling is mainly localized to the base of the nail.
3. Drumstick.

** Drumstick: It is a term used to describe the more advanced stages of digital clubbing. There is accumulation of connective tissue extends well beyond the base of the nail and involves the entire digit.

(b) Clubbing with periostosis:

® new-bone proliferation that accompanies digital clubbing.

- It is prominent in the long bones of extremities.

Other features:

1-    Symmetric arthritis-like changes.

2-    Coarsening of the subcutaneous tissue in the distal portions of arms and legs.

3-    Neurovascular changes in hands and feet (with chronic erythema, paresthesias, and increased sweating).

** Hypertrophic osteoarthritis(HOA) may be seen in:

-          Bronchogenic carcinoma.

-          Emypyema.

-          Lung abscesses.

-          Bronchiectasis.

* Thyroid Acropachy:

It is characterized by thickening of peripheral tissues. It occurs in 1% of patients with Graves’ disease and often is associated with exophthalmos and myxoedema of hands and feet. It resembles HOA in the sense that it is associated with digital clubbing and periosteal new bone formation. It involves hands and feet instead of long bones. Moreover it spares the joints and is usually painless.

Skin Examination

* Skin abnormalities with specific disease

(1) Amyloidosis:

-          Recurrent purpura; purpura in response to minimal trauma to skin as gentle pinch. This is due to fragile vascular wall, yellow or reddish brown papules.

-          Amyloidosis may be primary or secondary.

** Amyloidosis may be associated with:

• Restrictive cardiomyopathy.
• Postural hypotension.
• ECG: may show

-          Low voltage.

-          Conduction defects.

-          Abnormal Q-wave.

(2) Sarcoidosis:

- It is a granulomatous disease of unknown cause.

** Sarcoid skin lesions:

(a)Red, painful, tender nodules, of anterior portion of lower extremity.

(b)   Sarcoid papules with atrophic centers, around the nose and mouth.

(c)May be diffuse with serpiginous borders.

(d)   In some cases, plaques may develop and simulate psoriasis.

** Cardiovascular lesions:

- No direct correlation between skin lesion and systemic involvement however:

• Myocardium may be affected ® heart failure.
• Arrhythmias.
• Atrio-ventricular block.
• ECG ®           · A.V block.

· Q-wave.

(3) Thyroid dysfunction:

(a) Hyperthyroidism:

-          Skin is warm and smooth.

-          Palms are pink and moist.

-          Nails may show oncholysis.

-          Pretibial myxoedema ® flesh-colored plaques.

** Cardiovascular abnormalities:

·         Atrial fibrillation.

·         Hypermetabolic states may aggrevate heart failure and angina pectoris.

·         High-output heart failure.

(b) Myxoedema:

-          Skin is puffy, dry and swollen but does not pit with pressure.

-          Slightly yellow color to the skin (as the carotene is metabolized poorly by the liver).

-          Outer part of eyebrows may disappear and scalp hair may become brittle.

-          Axillary and pubic hair become sparse.

** Cardiovascular abnormalities:

-          Bradycardia.

-          Pericardial effusion.

-          Coronary heart disease.

-          Low-voltage ECG.

-          Myocardial disease.

(4) Systemic lupus erythematosus:

(a) Skin is highly sensitive to sunlight:

·         Persistent flush & urticaria.

·         Descoid lesion® scaly, reddish areas with follicular plugging.

·         Ulcers may develop.

(b) Malar rash:

-          A reddish macular eruption, but may be urticarial.

-          Usually, found over, nose & cheeks ® butterfly or bat wings appearance.

(c) telangiectasis, purpura, Raynaud’s phenomenon, subcutaneous nodules and paniculitis may develop.

** Cardiovascualr abnormalities:

-          Pericarditis.

-          Libmansacks disease: It is characterized by fibrous, or warty, lesions on the heart valves. (The undersurface of mitral valve, chordae tendineae & papillary muscles).

-          Embolic phenomenon.

-          Mitral and aortic regurgitation.

-          Coronary affection (lupus arteritis).

** Myocardial infarction is a common cause of death in such patients.

-          Rarely significant myocarditis.

(5) Scleroderma (Progressive systemic sclerosis):

· It is a connective tissue disease.

* Skin: It is tight, thick associated with vasculitis.

-          Raynaud’s phenomenon and ulcerations or scars of finger digits.

-          Skin of the face may be affected ® expressionless & immobile skin around mouth.

-          Telangectasia and hyperpigmentation.

** CREST syndrome: there are 4 out of 5 features are skin lesion:

            · Calcinosis ® C.

            · Raynaud’s phenomenon ® R

· Esophageal dysfunction ® E         

· Sclerodactyly ® S  

            · telangiectasia ® T

* Cardiovascualr abnormalities:

-          Myocardial fibrosis and conduction defects.

-          Coronary arteritis ® coronary spasm.

-          Angina pectoris and myocardial infarction.

-          Arrhythmias.

-          Pericarditis.

-          Pulmonary hypertension.

(6) Dermatomyositis:

·         Connective tissue disease.

- Muscle weakness before skin lesion:

·         Skin:

a) Transient lesions:

-          Violaceous macules on face, trunk and extremities.

-          Typically it involves eyelids.

-          Telangiectasia.

-          Scaly reddish lesion on joints.

b) Persistent lesions:

-          Gottron papules: small, flat violaceous palques overlie the interphalangeal joints of hands.

-          Nail folds ® red and glistening.

-          Small areas of pigmentation, and depigmentations.

-          Subcutaneous calcium deposition ® cutaneous ulcers.

-          Vesicles & bullae may develop.

* Cardiovascular abnormalities:

-          Less common.

-          Arrhythmias.

-          Conduction defects (especially complete AV- block).

(7) Diabetes mellitus:

a.     Diabetic dermopathy: pigmented atrophic scars in the pretibial areas.

b.     Bullous diabeticorum: large bulous filled with blood, heald without scars, in the hands, forearm, feet, & lower legs.

c.      Eruptive xanthomas: small pinkish yellow papules with a red base erupt on the buttocks and extensor surface of forearms (Diabetes & hypertriglyceridemia).

d.     Lipodystrophy: at the site of injection of insulin.

e.      Necrobiosis lipoidica diabeticorum: Small red papules over tibias ® gradually increase in size ® atrophic with red border and yellow center.

* Cardiovascular abnormalities:

·         Atherosclerosis.

·         Angina pectoris.

·         Myocardial infarction.

·         Incidence of heart failure is increased.

·         Diabetic cardiomyopathy.

·         Infant with diabetic mother ® may develop hypertrophic cardiomyopathy.

(7) Hyperlipidemia:

-Xanthomatous lesions: localized infiltration of lipid-containing macrophages, that are located within the tendons and skin.

-          Eruptive xanthomas.

-          Soft tuberous xanthomas.

-          Eye-lid xanthomas.

(8) Hyperesinophilic syndrome:

* Skin: - Red hyperpigmented papules or macules.

-          Uriticaria & angioedema.

-          Perifollicular papules.

-          Skin itches and scratch markers.

* Cardiovascilar abnormalities.

-          Myocarditis.

-          Arteritis of small coronaries ® CAD

-          Mural thrombi are common.

-          Heart failure.

(9) Endocarditis:

a)    Skin lesions such as infected wounds or furuncles that serve as a source for bacteremia and subsequent endocarditis.

b)   Skin lesion secondary to endocarditis:

·         Petechial hemorrhage of skin and mucous membrane.

·         Osler’s nodes.

·         Janeway lesions.

·         Splinter hemorrhage.

·         Finger clubbing.

(10) Hemochromatosis: may be primary (genetic) or secondary:

-          Excessive iron that deposits in tissues can cause cirrhosis, diabetes mellitus, artheritis, hypogonadism and cardiomyopathy.

* Skin:

            - may has a bronze color due to hypermelanosis.

- may be a salte-gray color due to hemosiderin.

-The skin may become dry and scaly.

-The hair may become sparse and spooning of nails may occur.

** Cardiovascular abnormalities:

-          Restrictive cardiomyopathy.

-          Heart failure.

-          Cardiac arrhythmias.

-          Low voltage QRS complexes of ECG.

(11) Multiple lentigines syndrome:

It is an inherited syndrome (LEOPARD): Lentigines, Electrocardiogrpahic abnormalities, occular hypertolerism, pulmonary valve stenosis, abnormalities of genitalia, retardation of growth, and deafness.

*        Skin: is covered with numerous small tan to brown macules. These lesions are present at birth or soon after birth and increase as the child grows.

* Cardiovascular lesions:

-          Pulmonary valve stenosis.

-          Sometimes aortic valve stenosis.

-          Obstructive cardiomyopathy.

-          Endocardial fibrosis.

-          Left atrial myxomas.

-          Conduction defects.

(12) Carcinoid syndrome:

* Skin:

-          Transient flush.

-          Perminant changes in capillaries of skin ® telangiectasia ® bluish red appearance.

-          Sometimes, hyper-pigmented scally dermatitis.

-          Sometimes scleromatous changes.

* Cardiovascular lesions:

-          Tricuspid stenosis, or regurgitation.

-          Pulmonary regurgitation.

(13) Behcet’s syndrome:

-          Vasculitis of skin.

-          Uveitis.

-          Ulcerations of mucous membrane of mouth and genitalia, of both sexes.

-          Synovial membrane inflammation.

-          Development of papule or pastule after a needle prick.

** This syndrome is accompanied by:

-          Pericarditis.

-          Myocarditis.

-          Thrombophlebitis or deep venous thrombosis.

-          Aneurysms or thrombosis of major arteries.

(14) Reiter’s syndrome: may be

-          Sexually transmitted disease.

· Dermatitis.

· Conjunctivitis.

· Urethritis. &

· Polyarthritis.

* ® small vesicles and papules with red base of palms, soles and nail beds.

-          Vesicle with a red base located near urethral meatus

-          A crusted lesion may appear on glans penis..

-          Oropharyngeal ulcers.

* Cardiovascualr leisons:

- Pericarditis.

- Conduction defects.

- Aortitis (obstruction of vasa vasorum) ® aortic regurgitation.

- Affection of mitral annulus ® mitral regurgitation.

(15) Malignant Melanoma:

            It is a cutaneous mole that changes in color, size, shape, or consistency, or mole that itches or become painful.

* Cardiovascular lesions:

-          Cardiac metastasis.

-          Charcoal heart.

-          Pericarditis & effusion ® tamponade.

-          Arrhythmias.

-          Conduction defects.

-          Heart failure may occur.

(16) Rheumatic fever:

·         Subcutaneous nodules: non-tender, non-movable nodules occur on elbows, the forehead and bony prominences.

·         Erythema marginatum: is a pin-black, with clear center, and occurs on abdomen, trunk and proximal parts of legs & arms.

* N.B.: Other causes of subcutaneous nodules & erythema marginatum.

-          Rheumatoid arteritis.

-          Lupus erythematosus.

-          Annular erythema may occur in glumerulonephritis, drug toxicity & sepsis.

(17) Rheumatoid arthritis:

a.     Early: transient rash of pink macules & papules on the face, palms & soles in association with, low grad-fever, months or years before artheritis.

b.     Late: in adults there are rheumatoid nodules, these are firm, movable subcutaneous nodules, (one to several centimeters in size) & are painless. They are found on the bony prominances.

c.      Palmar erythema, purpura ulceration of skin due to arteritis.

d.     Raynaud’s phenomenon may occur.

* Cardiovascular lesions:

-          Pericarditis & pericardial effusion.

-          Aortic & mitral regurgitation.

-          Myocarditis (rare) & coronary arteritis.

-           Cardiomyopathy                   - Conduction defects.

(18) Polyarteritis nodosa:

- It is a type of vasculitis, involves all layers of small and medium size arteries.

* Skin lesions:

-          Erythema.

-          Vesicular eruptions.

-          Urticaria.

-          Erythema nodosum.

-          Macular & papular rashes.

-          Gangrene of fingers and toes may develop.

Cardiovascular lesions:

-          Hypertension.

-          Left ventricular hypertrophy.

-          Pericarditis.

-          Coronary artery disease.

-          Epicardial coronary arteries may dilate and aneurysm formation.

-          Conduction defects.

(19) Kawasaki disease:

            It is a childhood disease of unknown etiology:

* Skin lesions:

(a) 5-15 days:

-          Red nodules.

-          Edema of feet and hands.

-          Palms and soles become purplish-red.

(b) Two to 3 weeks:

· Desquamations.

(c) Week later: transverse lines in the nails.

* Cardiovascular lesions:

-          Pericarditis.

-          Myocarditis.

-          Conduction defects.

-          Mitral regurgitation, due to papillary muscle dysfunction.

-          Coronary artery aneurysm and stenosis, occur in one fifth of patients.

-          Coronary spasm.

-          Coronary microemboli.

(20) Neurofibromas:

-          Café-au-lait spots and axillary freckles (Crowe’s sign) of Van Recklinghausen’s disease.

-          It is associated with (pheochromocytomas).

(21) Hemochromatosis:

-          Bronzing color.

-          The patient is also diabetic.

-          It is associated with restrictive cardiomyopathy.

(22) Telangiectasias  of Osler-Weber-Rendu syndrome:

-          It is associated with pulmonary arterio-venous fistulas in about of 16% of cases.