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.

Percutaneous closure of atrial septal defect using left internal jugular vein access in a child with situs inversus and absence of inferior caval vein.

Femoral vein access is the first choice for percutaneous atrial septal defect closure, and when it cannot be used due to anatomic reasons, the alternative sites should be considered, frequently increasing the complexity of the procedure. Here we report the case of a 3-year-old boy, with situs inversus and dextrocardia, electively referred for percutaneous closure of an ostium secundum atrial septal defect. During the procedure, agenesis of the infra-hepatic segment of the inferior caval vein was diagnosed, and no double inferior caval vein or right superior caval vein were identified by ultrasound or angiography.

Adenosine for vasoreactivity testing in pulmonary hypertension: a head-to-head comparison with inhaled nitric oxide.

Background: APVT is an invasive method recommended for symptomatic patients with PAH that permits the identification of the minority of patients (<20%) that may benefit from long-term calcium channel blockers. Adenosine has been indicated in guidelines as a vasodilator agent of choice for APVT, although it has not been directly compared with iNO, the gold standard for this test. The objective of the study was to compare adenosine with inhaled nitric oxide (iNO) for acute pulmonary vasoreactivity testing (APVT) in pulmonary arterial hypertension (PAH), in order to determine the efficacy and safety of the first in the clinical setting.

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.