Pulmonary hypertension, in association with congenital heart diseases, is seen in large systemic-to-pulmonary communications such as ventricular septal defect, patent ductus arteriosus, etc. Certain cyanotic congenital heart diseases with large pulmonary blood flow also have pulmonary hypertension. Pulmonary vascular disease, secondary to pulmonary hypertension, may develop if the defect is not repaired in infancy or early childhood. Progressive pulmonary vascular disease leads to irreversible changes in the vessel wall with reversal of shunt and this condition is termed Eisenmenger syndrome. Once Eisenmenger syndrome develops, repair of congenital heart disease is contraindicated. In those with borderline changes in pulmonary vasculature, a comprehensive assessment must be made to test for operability. This assessment should include cardiac catheterization for obtaining hemodynamic data in basal state and after challenge with vasodilators. Lung biopsy may be needed in select cases and should be performed if expertise is available for histological interpretation of the biopsy specimen. Patients with pulmonary hypertension and congenital heart disease should undergo repair of the defect in time, usually in the first year of life. Once Eisenmenger syndrome sets in, the treatment is generally supportive. Pulmonary vasodilators, such as sildenafil, bosentan, have been found to be beneficial in some cases of Eisenmenger syndrome. Lung or heart/lung transplantation should be reserved for severely symptomatic cases. The natural history of patients with Eisenmenger syndrome is considerably better than that of idiopathic pulmonary hypertension. Over half of the patients survive for more than 50 years. Pregnancy and noncardiac surgery may adversely affect the course of the disease.

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Pulmonary hypertension (PH) is a life-threatening disease characterized by a marked and sustained elevation of pulmonary artery pressure. The disease results in right ventricular failure and death. The pathogenesis of PH is complex and multifactorial. Vasoconstriction, remodeling of the pulmonary vessel wall and thrombosis contribute to increased pulmonary arterial pressure in PH. The current therapeutic approaches (prostacyclin analogs, endothelin-receptor antagonists, and phosphodiesterase type 5 inhibitors) for the treatment of PH mainly provide symptomatic relief, as well as some improvement of prognosis. However, they do not appear to reverse or modify the disease. Recently, several growth factors and growth factor receptor tyrosine kinases (RTK) have been implicated in the abnormal proliferation and migration of pulmonary arterial smooth muscle cells, including platelet-derived growth factor (PDGF), epidermal growth factor (EGF), fibroblast growth factor (FGF) and vascular endothelial growth factor (VEGF) and others. Hence, this review describes the potential role of RTKs in pulmonary vascular remodeling and emergence of RTK inhibitors as promising therapeutic targets.

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The authors provide histopathological images of pulmonary vascular lesions from patients with diseases of several etiologies and clinically presenting with pulmonary hypertension. The first part of this article was published in the PVRI REVIEW Vol 1, Issue 1, January 2009). In this second part, the discussion and images are about specific vascular lesions, which include the following:

1. Obstruction to pulmonary venous drainage
2. Pulmonary occlusive venopathy
3. Pulmonary microvasculopathy (formerly, capillary hemangiomatosis)
4. Chronic thromboembolic pulmonary vascular lesions
5. Pulmonary arterial obstruction by parasites or their eggs
6. Pulmonary tumor thrombotic microangiopathy

The sources were lung biopsies obtained for pre-surgical evaluation in children with congenital heart disease, lung explants in cases of lung transplantation and lung tissue from necropsies.

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Monge's disease or Chronic Mountain Sickness is characterized by an excessive polycythemia (hemoglobin >21 g/dl in men and >19 g/dl in women) in high-altitude dwellers, with a prevalence of 5-18% above 3200 m. Clinical signs include headache, fatigue, sleep disturbances, dyspnea, dizziness, tinnitus, paresthesia and digestive complains. Pulmonary hypertension is frequently associated, which might lead to congestive heart failure. Hypoxemia due to alveolar hypoventilation may play a central role in the over-stimulation of erythropoiesis leading to increased red cell mass and blood viscosity, systemic and pulmonary hypertension. Acetazolamide is the first efficient pharmacological treatment of Monge's disease without adverse effects, by reducing nocturnal hypoventilation and blunting erythropoiesis. Its low cost might allow a wide development with a considerable positive impact on public health in high-altitude regions. Further studies are necessary to evaluate the prevalence and importance of pulmonary hypertension and its consequences on cardiac function.

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