Chapter 14

 

ESSENTIAL PULMONARY HYPERTENSION

    This is a rare situation more frequent in female than in male patients.
    The main pathology is located in the arteriola with enlargement and hypertrophy of the medial in conjunction with hyperplasia of the intima. Hypoxia will be seen in the late stages of this disease with subsequent increase of the pulmonary hypertension.
    Right ventricular hypertrophy is seen with right atrial enlargement. Dilatation of the pulmonary artery is present. Atrial septal defect with right to left shunts may be observed in the last stages.
    The diagnosis of essential pulmonary hypertension is in general done by exclusion and many times cardiac catheterization is necessary to demonstrate the total absence of any other circumstances leading to such a clinical picture.

SYMPTOMS

    The patient's symptoms will vary depending uon the stage of the disease. The most common ones being palpitations, general weakness and dyspnea with signs of right ventricular failure. Right upper quadrant abdominal tenderness is not unusual with peripheral ankle edema and occasionally ascites.

PHYSICAL EXAMINATION

    The physical examination revealed a loud S2 with a systolic ejection murmur in the pulmonary valve area and a high pitched early diastolic murmur of pulmonary regurgitation. If the main pulmonary artery pressure is higher than 60 mm Hg, the second sound may be palpable and the dilatation of the pulmonary artery palpated at the second and third left intercostal spaces.
    If the patient presents right ventricular failure, jugular engorgement and hepatomegaly may be noted.
    In severe cases, A wave in the jugular pulse is seen. Occasionally, right to left shunt may appear in time through a patent foramen ovale.
    Mild cyanosis may be seen in relation to exertion which can be permanent in cases of systemic pulmonary hypertension.

ELECTROCARDIOGRAM

    Electrocardiogram will show signs of right ventricular hypertrophy with marked right axis deviation (right ventricular systolic overload). R to qR complex will be present in V1, V2 and V3 with deep inversion of the T wave in the same leads in relation to the degree of right ventricular hypertrophy. P pulmonale is observed in 75 % of the cases with occasional bilateral atrial overload in case of patent foramen ovale.

ROENTGENOGRAM

    The roentgenogram will demonstrate:
      Dilatation of the pulmonary artery
      Prominent hilar vessels
      Clear peripheral lung fields
      Right ventricular hypertrophy which is better observed in lateral view

    In any event, there are no typical signs to differentiate essential pulmonary hypertension from any other kind of systemic pulmonary hypertension, as for example, Eisenmenger's Syndrome.
    The cardiac catheterization will be necessary to demonstrate the severity and hemodynamic consequences of the pulmonary hypertension and to rule out intracardiac shunts. In such regard, the angiocard iogram is essential as dye dilution curves in order to entirely certify the absence of ventricular septal defect, ASD or PDA.
    In summary, this entity can only be diagnosed in a patient with a normal pulmonary wedge pressure and elevation of the pulmonary artery pressure with total absence of occlusive lesions in the pulmonary artery branches and absence of extracardiac shunts. The syndrome is completed with the findings of right ventricular hypertrophy.
    The symptoms in these patients frequently appear only when the cardiac output is fixed due to the marked pulmonary hypertension. In this regard the finding of a patient with no previous history of cardiac problems, murmur, or rheumatic heart fever and with marked pulmonary hypertension of recent onset, will strongly suggest the diagnosis of primary pulmonary hypertension.
    In the differential diagnosis, the observance of clubbing of the fingers should strongly suggest the possibility of Eisenmenger's complex against pulmonary hypertension, where clubbing is rare.

DIFFERENTIAL DIAGNOSIS

    The differential diagnosis should be made with:

  1. Recurrent pulmonary embolism and consequent pulmonary hypertension
  2. Chronic obstructive and restrictive lung diseases
  3. Severe mitral stenosis with pulmonary hypertension
  4. Eisenmenger's Syndrome