Cyanosis after Kawashima-Fontan: hybrid approach to mixing and matching flows.

Surgical approaches to a single ventricle with absent infrahepatic segment of the inferior vena cava include a bidirectional Glenn and separate hepatic venous connection to the pulmonary arteries. If sites of insertion into the pulmonary arteries are widely separated, hepatic flow is directed to only one lung. Pulmonary arteriovenous malformations may form in the contralateral lung and result in severe cyanosis. We report a case of resolution of pulmonary arteriovenous malformations after successful rerouting of hepatic venous flow to the hemi-azygous vein in a 16-year-old patient. A dilatable pulmonary artery band reduced cyanosis by limiting flow to the affected lung until resolution of pulmonary arteriovenous malformations.

Surgery for anomalous aortic origin of the coronary artery.

BACKGROUND: Anomalous aortic origin of the coronary artery (AAOCA) has been associated with coronary ischemia, myocardial infarction, and sudden death. Advances in echocardiography and computed tomography have identified at-risk patients. Treatment options include unroofing strategies in symptomatic and asymptomatic patients. We review our experience for efficacy and safety. METHODS: Between 1998 and 2008, we performed coronary unroofing in 22 patients with AAOCA without aortic commissural detachment. Of 7 patients with "left from right" AAOCA, 4 had chest pain only, 1 had syncope, 1 had myocardial infarction, and 1 was asymptomatic. Of 15 patients with "right from left" AAOCA, 11 had chest pain only, 4 had syncope, and none were without symptoms. Median age was 15 years (range, 5 to 54). Eight patients had concomitant procedures, most commonly patent foramen ovale closure. RESULTS: There were no deaths or complications. Mean cross-clamp time was 53 minutes. Mean length of stay was 4 days. Postoperative evaluation included echocardiography, computed tomography angiogram, stress thallium, stress echocardiography, and exercise stress test. In all patients, the repaired coronary was patent, with demonstrated flow. Mean follow-up was 17 months (range, 1 to 63). CONCLUSIONS: Anomalous aortic origin of the coronary artery is emerging as an identifiable disease entity associated with symptoms or sudden death. We conclude that coronary unroofing is a safe and effective therapy for symptomatic patients. Surgery for asymptomatic patients has been more controversial, with growing advocacy for patients with "left from right" as opposed to "right from left" origins. Prospective studies will be required to answer these questions.

Partial prolapse of a HELEX device associated with early frame fracture and mitral valve perforation.

One or more breaks in the wire frame of the HELEX Septal Occluder (W.L. Gore and Associates, Flaggstaff, Arizona) were identified in 5% of patients in the multicenter pivotal trial leading to FDA approval. Fractures of the perimeter frame have not previously been associated with any apparent deleterious clinical effects. We report a case in which the broken ends of wire at a frame fracture were located in a position that resulted in perforation of the anterior mitral valve leaflet. There was significant mitral regurgitation and the device was removed surgically. The possible etiologies and prevention of wire frame fractures are also discussed.

Melbourne shunt promotes growth of diminutive central pulmonary arteries in patients with pulmonary atresia, ventricular septal defect, and systemic-to-pulmonary collateral arteries.

BACKGROUND: We manage patients with pulmonary atresia, ventricular septal defect, major systemic-to-pulmonary collateral arteries, and diminutive central pulmonary arteries with a staged approach. The first procedure is a central end-to-side aortopulmonary shunt (Melbourne shunt) intended to cause growth and development of the central pulmonary arteries. We subsequently measured central pulmonary artery growth after Melbourne shunt. METHODS: Forty consecutive patients were followed after Melbourne shunt. The maximum pulmonary artery diameter was measured at the time of surgery and at subsequent catheterizations or surgery. RESULTS: Median pulmonary artery size at surgery was 2 mm. The median pulmonary artery diameter was 5.5 mm at first assessment (median, 6.35 months) and 7 mm at most recent assessment (19.7 months). Mean modified Nakata index increased from 27 mm(2)/m(2) at surgery to 138 mm(2)/m(2) at first assessment, and 176 mm(2)/m(2) at final assessment. There was one acute shunt failure from anastomotic stenosis. Thirteen patients (32.5%) required 21 percutaneous interventions. There were 4 deaths during a median follow-up of 68 months. At the time of complete repair (n = 25) all patients required pulmonary artery augmentation, and 8 are in various stages of palliation. The remaining patients are considered not reparable owing to irreversible pulmonary hypertension (n = 4) or have required fenestration of ventricular septal defect after complete repair (n = 2). CONCLUSIONS: Melbourne shunt promotes modest growth of central pulmonary arteries leading to complete repair in the majority of patients. There is considerable need for further interventions in these patients to augment the size of the pulmonary arteries.

Transhepatic Broviac catheter placement for long-term central venous access in critically ill children with complex congenital heart disease.

OBJECTIVE: Critically ill children with cardiac disease often require prolonged central venous access. Thrombosis of systemic veins or the need to preserve vessels for future cardiac procedures limits sites for placement of central venous catheters in these patients. This study evaluates the use of Broviac placement via the transhepatic approach for this patient population. DESIGN: A retrospective review. SETTING: A tertiary care center. PATIENTS: All children with complex congenital heart disease who underwent transhepatic Broviac placement between May 2000 and April 2004. INTERVENTIONS: Transhepatic Broviac placement. MEASUREMENTS AND MAIN RESULTS: Thirty-two children with a median age of 5 months (20 days-5.3 yrs) and a median weight of 4.2 kg (2.2-24.9 kg) underwent 40 transhepatic Broviac placements. There were three (8.8%) procedural-related complications. One patient suffered an intra-abdominal bleed requiring an urgent laparotomy and removal of the Broviac, one patient required transfusion because of a mild self-contained intra-abdominal bleed, and one patient developed temporary complete heart block. There was one catheter infection. Thrombus was noted by echocardiography on the tip of two Broviacs; however, no intracardiac vegetations or embolic events occurred. There was no mortality related to the procedure. Broviacs remained in place for a median of 36 days (1 day-6 months). Five Broviacs were dislodged inadvertently (two during cardiac massage and three resulting from patient manipulation). The remaining Broviacs were electively removed safely without coil embolization. At a median follow-up of 3.5 months (10 days-3 yrs), there have been no long-term complications related to the Broviacs. CONCLUSIONS: Transhepatic Broviac catheters can be used safely in critically ill children with cardiac disease and remain indwelling for adequate periods of time. This modality of prolonged vascular access should be considered for children whose veins are occluded or need to be preserved for future procedures.

HLHS with severe aortic insufficiency in a patient with 45,X/46,XY mosaicism.

Aortic insufficiency is not a part of the hypoplastic left heart syndrome. This report describes a rare case of congenital aortic insufficiency from a detached leaflet in a patient with hypoplastic left heart syndrome and 45,X/46XY mosaicism. The patient was subsequently treated with the modified Norwood procedure along with suture closure of aortic valve.