Background: The effectiveness and safety of macitentan, an endothelin-receptor antagonist (ERA) in the treatment of pulmonary arterial hypertension (PAH), has been demonstrated in numerous randomized clinical trials including SERAPHIN, focused on the reduction of morbidity and mortality. Objectives: Our aim was to demonstrate the clinical and echocardiographic progression using macitentan in Indian patients with PAH. Settings and Design: It was a retrospective study of 20 patients with multiple etiologies of PAH who had begun macitentan in routine clinical practice from a single center. There were 55% of patients with existing PAH therapies. Subjects and Methods: The World Health Organization functional class (WHO-FC), 6-min walking distance, N-terminal prohormone of brain natriuretic peptide level (NT-pro-BNP), and echocardiographic data such as tricuspid annular plane systolic excursion (TAPSE), systolic pulmonary artery pressure (sPAP), and the occurrence of pericardial effusion were collected at baseline and 12-month follow-up. The statistical analysis was performed using SPSS software. Results: Of the 20 PAH patients, 70% were women. The majority were in WHO FC II (50%), while 35% were in FC III and 15% were in FC IV. The mean age was 43.4 years at the start of the therapy with macitentan. After 6 months of macitentan therapy, 85% showed substantial improvement in their FC, each increased its 6-min walking distance test ( P < 0.0001), and there was a significant reduction in plasma levels of NT-pro BNP ( P < 0.0001). There has also been an improvement in echocardiographic parameters such as TAPSE and sPAP ( P < 0.0001). Conclusions: Our findings indicate that macitentan has been well tolerated and beneficial in Indian patients with PAH and further, future research is required to verify these results.

An anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA), a rare congenital anomaly, the enlarged right coronary artery supplies the left ventricle through retrograde collaterals before preferentially directing into the lower-pressure pulmonary artery system, resulting in coronary steal. The infant type is characterized by the absence of collateral vessels, and global myocardial ischemia is a major cause of death in infancy. If not treated, up to 90% of them will die during the 1st year of life. Myocardial ischemia or infarction left ventricular (LV) dysfunction with or without mitral regurgitation, life-threatening arrhythmias, or sudden cardiac death may all be manifestations of symptomatic adult-type ALCAPA. In those patients who have survived to adulthood without surgery, pathophysiological mechanisms enabling adequate LV perfusion, such as a large number of well-formed functioning collaterals, and in a few cases, the presence of ostial stenosis of the left coronary artery (LCA), provide selective survival advantage. We review the correlation between clinical presentation, pathophysiological findings, and angiographic features of ostial stenosis of LCA in the setting of adult-type ALCAPA.