He study of primary pulmonary hypertention, clinical, echocardiography and catheterization assessement

الملخص EN

Abstract BACKGROUND: A Retrospective study of 31 patients who diagnosed as a case of primary pulmonary hypertension (PPH) in Ibn-Albitar hospital for cardiac surgery, from July 1999 to April 2007, to study the clinical pattern of PPH in children and adult, who diagnosed as acase of PPH clinically and echocardiographically and confirmed by diagnostic cardiac catheterization.

Patient and Method: This study excluded cases of secondary PAH and those without catheterization.

Two cases excluded from PPH cases (one patent ductus arteriosus-eisenminger`s syndrome and other is large sinus venosus atrial septal defect).

These data were collected from medical files or catheterization files, including:(age, gender, duration of symptoms before reaching diagnosis of PPH, number of hospital admissions, signs, and symptoms of PPH, echocardiographic findings), considering pediatric age group ≤ 18year and adult >18year, and the catheterization data were: way of reaching final diagnosis in catheterization, severity of PAH and mean of MRAP, MPAP) also morbidity and mortality.

Conclusions :the pediatric cases tend to present earlier than adult cases, Females cases more than males cases.

The duration of symptoms before catheterization was short, and it was shorter in adult cases.

The most common symptoms in pediatrics are(cyanosis, fatigability, and extensional dyspnea), while in adult (exertional dyspnea, chest pain and fatigability).The most common signs in pediatrics are (normal physical examination, loud second heart sound and murmur)while in adult (murmur, dyspnea, loud second heart sound).

The catheterization approach to reach definite diagnosis in most pediatrics (by normal pulmonary capillary wedge pressure) while in adult (by exclusion secondary causes).

Recommendations: atrial septal defect-sinus venosus should be considered in exclusion list of secondary PAH, familial cases need genetic analysis, loud second heart sound is important clue for PPH, especially if associated with dyspnea on exertion.

Pregnancy still wanted by certain families, and counseling for patients and their families regarding risk of pregnancy is essential.