Reverse Potts for the Treatment of Severe Idiopathic Pulmonary Hypertension in Children.

Idiopathic pulmonary arterial hypertension is a rare and progressive disease with poor prognosis. Many patients progressively worsen even when using combinations of specific drugs for its treatment. Herein, we present our experience in the management of three children with severe pulmonary arterial hypertension refractory to clinical treatment who underwent Potts surgery in addition to clinical treatment.

Testing pulmonary vasoreactivity.

Pulmonary arterial hypertension is classified as idiopathic or secondary (associated with collagenoses, heart disease, portal hypertension, pulmonary thromboembolism, and pulmonary vascular diseases). Pulmonary vasoreactivity should be tested in order to define the best treatment option. Of the many drugs that have been used to test pulmonary vasoreactivity, inhaled nitric oxide is the best choice, due its specific pulmonary effect and very short half-life (5-10 s).

[Percutaneous closure of atrial septal defect using transhepatic puncture].

The percutaneous closure of ostium secundum (OS) atrial septal defect (ASD) is a well-established procedure and is today considered the treatment of choice due to its good results and low morbidity and mortality. The procedure is routinely performed through the inferior vena cava (IVC). However, this route of access is not always available due to the obstruction or congenital absence of the IVC.

[Sildenafil in the management of idiopathic pulmonary arterial hypertension in children and adolescents].

Objective: This study aims to provide data on the use of oral sildenafil in patients in New York Heart Association functional class III or IV with severe idiopathic pulmonary arterial hypertension unresponsive to conventional therapy.

Method: In this series, six patients with idiopathic pulmonary arterial hypertension were prospectively treated with 2 to 8 mg of oral sildenafil in four to six doses a day. All patients were submitted to physical examination, electrocardiogram and echocardiogram, chest computed tomography, ventilation and pulmonary perfusion scintigraphy, coagulation studies, and tests for collagen vascular disease, acquired immune deficiency syndrome and schistosomiasis in order to rule out secondary causes of pulmonary arterial hypertension.