Pulmonary hypertension refers to a group of diseases that is characterised by high pressure in the pulmonary artery, and pulmonary vascular resistance. Persistent pulmonary hypertension in the newborn is a condition in which the pulmonary artery pressure does not decrease after birth. It may occur in as many as 6.8 in 1 000 live births. Phosphodiesterase type 5 (PDE5) is the predominant phospho...

Misalignment of the pulmonary veins with congenital alveolar capillary dysplasia, although rare, has been reported as a cause of persistent pulmonary hypertension of the newborn. Reported cases have been mainly sporadic. Familial occurrence has been reported in only three instances. We present affected sibs with this condition. In addition to pulmonary abnormalities, urogenital abnormalities, i...

Pulmonary arterial hypertension (PAH) management has been transformed in recent times with the advent of cheap and effective diagnostic tools and therapy. Sildenafil, a phosphodiesterase-V inhibitor, has been at the centre of this treatment, and its success in treating PAH has led to its widespread uptake in adult and paediatric pulmonary hypertension (PH), as a first line treatment choice. Thi...