| 1.1 |
Idiopathic/Primary |
Symptomatic treatment to prevent right ventricular failure and hypoxemia Avoid conception if possible Hospital admission in second trimester ICU care for decompensation and in third trimester. Early cardiologist care during antenatal visits, labor, delivery, and postpartum period
|
| 1.2 |
Heritable |
Includes familial and simplex PAH Autosomal dominant inheritance Mean survival is 2.8 years after diagnosis Significant stress of pregnancy in HPAH with high maternal mortality rates Early cardiologist intervention, monitoring of right ventricular function, genetic studies Use of suitable contraceptive measures
|
| 1.3 |
Drugs and Toxin induced |
Exposure to appetite suppressants like aminorex, fenfluramine derivatives, benfluorex (withdrawn from the market) Amphetamines, phentermine, mazindol, Dasatinib, and interferons associated with PAH Identifying and stopping the implicating agent are vital for survival and reversibility Counseling regarding contraception and psychological support
|
| 1.4 |
Associated with |
• Treatment of connective tissue disease with disease-modifying agents and antiretroviral therapy will need modification in pregnancy to prevent fetotoxicity |
| 1.4.1 |
Connective tissue diseases |
• Concurrent pulmonary hypertension in these patients heralds a poor feto-maternal prognosis during pregnancy and labor. |
| 1.4.2 |
HIV infection |
• A multidisciplinary team approach with early involvement of cardiologist, rheumatologist, physician, and obstetrician |
| 2 |
PAH associated with heart disease (left ventricular dysfunction, congenital heart disease, valvular heart disease, cardiomyopathy) |
Optimization of the primary cardiac disease ICU admission for cardiac failure, arrhythmias, hypoxia, Eisenmenger syndrome Infective endocarditis prophylaxis Pulmonary vasodilators under the direct supervision of a cardiologist
|
| 3 |
PAH associated with lung disease or hypoxia |
Detected on chest examination, pulmonary function testing, and high-resolution computed tomographic lung imaging The unfavorable feto-maternal outcome in these cases with pregnancy due to poor cardiorespiratory reserve and pregnancy-induced stress Patients to continue home oxygen therapy along with medications for their lung disease Early involvement of cardiologist, pulmonologist, and intensivist (especially if ventilatory support is required)
|
| 4 |
PAH due to pulmonary embolism or diseases of large pulmonary vessels |
Screening is done by lung perfusion scanning Minimize radiation exposure for investigations during pregnancy In chronic thromboembolic pulmonary hypertension (CTEPH), surgical thrombo-endarterectomy may be considered before conception Early involvement of cardiologist and cardiothoracic vascular surgeon in care Consider DVT prophylaxis as per institutional protocol.
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