[Pulsatile Fontan: transcatheter closure of patent pulmonary artery. Follow up mid-term]. / Fontan pulsátil: oclusión transcatéter de la arteria pulmonar permeable. Seguimiento a mediano plazo.

OBJECTIVE: We report the percutaneous closure of the pulmonary artery with residual shunt in patients with Fontan type circuit. METHOD: Patients aged 9 and 11 years, with SaO2 of 88 and 96%, respectively. One of them coursing with headaches and functional class II. Both patients with total cavopulmonary anastomosis and fenestrated extracardiac conduit and permeable pulmonary artery (pulsatile Fontan). An Amplatzer duct-occluder device was implanted in the pulmonary artery entering from the femoral vein. Follow-up by means of clinical examination, imaging, and catetherization was pursued. RESULTS: Case 1, patency fenestration, Qp/Qs: 0.7/1. Case 2, closed fenestration, Qp/Qs; 1.3/1. We obtained immediate occlusion with 6/4 and 8/6 devices, respectively; pressure recordings revealed modification of the arterial morphology to biphasic; pulmonary pressure dropped 2 mm Hg in the first patient, without alteration in the second case; no changes in SaO2 were registered. Time of fluoroscopy was 57 and 45 minutes, respectively. Follow-up was maintained for 2.8 and 2.3 years, respectively. In patient 1, headaches disappeared and the fenestration was occluded with an Amplatzer septal-occluder one year later, raising SaO2 to 96%; no complications occurred nor was recanalization of the pulmonary artery needed in either case. CONCLUSIONS: Percutaneous occlusion of patent pulmonary artery in patients with Fontan type circuit is a feasible and effective procedure, and avoids overload of the single ventricle.

Fontan pulsátil: oclusión transcatéter de la arteria pulmonar permeable. Seguimiento a mediano plazo / Pulsatile Fontan: transcatheter closure of patent pulmonary artery. Follow up mid-term

OBJECTIVE: We report the percutaneous closure of the pulmonary artery with residual shunt in patients with Fontan type circuit. METHOD: Patients aged 9 and 11 years, with SaO2 of 88 and 96%, respectively. One of them coursing with headaches and functional class II. Both patients with total cavopulmonary anastomosis and fenestrated extracardiac conduit and permeable pulmonary artery (pulsatile Fontan). An Amplatzer duct-occluder device was implanted in the pulmonary artery entering from the femoral vein. Follow-up by means of clinical examination, imaging, and catetherization was pursued. RESULTS: Case 1, patency fenestration, Qp/Qs: 0.7/1. Case 2, closed fenestration, Qp/Qs; 1.3/1. We obtained immediate occlusion with 6/4 and 8/6 devices, respectively; pressure recordings revealed modification of the arterial morphology to biphasic; pulmonary pressure dropped 2 mm Hg in the first patient, without alteration in the second case; no changes in SaO2 were registered. Time of fluoroscopy was 57 and 45 minutes, respectively. Follow-up was maintained for 2.8 and 2.3 years, respectively. In patient 1, headaches disappeared and the fenestration was occluded with an Amplatzer septal-occluder one year later, raising SaO2 to 96%; no complications occurred nor was recanalization of the pulmonary artery needed in either case. CONCLUSIONS: Percutaneous occlusion of patent pulmonary artery in patients with Fontan type circuit is a feasible and effective procedure, and avoids overload of the single ventricle.

Double-orifice mitral valve with intact atrioventricular septum: an echocardiographic study with anatomic and functional considerations.

We identified 18 patients with double-orifice mitral valve (DOMV) and intact atrioventricular (AV) septum out of 40,179 echocardiographic studies performed between 1997 and 2002 at Children's Hospital, Denver, CO. In this study we describe (1) the anatomic characteristics of the DOMV in the absence of AV septal defect, (2) the function of the mitral valve by spectral and color Doppler flow mapping, and (3) associated lesions. The topographic location of the orifices in the leaflets suggests possible embryologic mechanisms of DOMV. In this series, DOMV was most commonly associated with left-sided obstructive lesions (in 39% of patients). Spectral and color Doppler interrogation demonstrated a normal flow profile in most cases; only 2 patients had significant mitral regurgitation or stenosis. Therefore, due to the uncertain natural history of this lesion and the potential need for endocarditis prophylaxis, careful imaging of the mitral valve is recommended, particularly in the presence of left-sided obstructive lesions.

Mixed total anomalous pulmonary venous connection: Case report with bilateral venous collectors.

We present a case report of a 3-month-old boy with a mixed total anomalous pulmonary venous connection. The patient had situs solitus, small atrial septal defect, and 2 separate venous collectors. The right pulmonary veins drained through a right-sided venous collector into the coronary sinus. The left-sided pulmonary veins drained through the left-sided venous collector directly into the right superior vena cava. The use of the echocardiogram and Doppler color flow mapping to establish a detailed morphologic analysis, the sites of connection, and the presence of pulmonary venous obstructions as well as the value of this information to facilitate a successful surgical repair are discussed.