Primary resection of Kommerell's diverticulum in a 37-year-old patient.

A previously non-operated 37-year-old patient presented with esophageal dysphagia for solid food and stridorous breathing. Computed tomography and magnetic resonance showed a 33-mm wide Kommerell's diverticulum. The diverticulum and the obliterated ductus arteriosus arising from it towards the pulmonary artery were resected. The left subclavian artery, which originated from the diverticulum, was reimplanted into the descending aorta. In addition to dissecting any tissue which is making vascular ring-like structures around the trachea and the esophagus, it is important to resect the diverticulum itself and reimplant the left subclavian artery arising from it, in this way leaving the aortic arch free from any additional dislocating forces.

Outcome after surgical repair/palliation of congenitally corrected transposition of the great arteries.

BACKGROUND: The surgical concepts for patients with congenitally corrected transposition of the great arteries (CCTGA) address discordant connections and associated lesions. The outcomes after biventricular repair without correction of discordant connections ("classic repair", or with its correction "anatomic repair") and after "univentricular palliation" were investigated. METHODS: All patients with CCTGA who underwent "classic repair" (n = 39), "anatomic repair" (n = 6), or "univentricular palliation" (n = 11) between 1978 and 2006 were analyzed. The most frequently associated lesions were ventricular septal defect (n = 48), tricuspid insufficiency (TI) (n = 20) and functionally single ventricle (n = 11). RESULTS: Thirty-day mortality was 4 % (2/56). Mean follow-up for early survivors was 7.2 +/- 7.1 years. Eight patients died late, two after heart transplantation. Survival was not significantly different between patients who underwent "anatomic" or "classic repair", or "univentricular palliation": 83.3 +/- 15.2 %, 79.7 +/- 6.9 %, 90.9 +/- 8.7 % at 10 years, respectively. In multivariate analysis, the presence of TI emerged as the only risk factor for late death ( P = 0.004). Twenty patients required reoperation, mainly for TI (n = 10) and conduit failure (n = 6). Freedom from reoperation was lower after "anatomic repair", but ventricular function was better and atrioventricular valves were more competent than after "classic repair". CONCLUSIONS: Biventricular "anatomic" or "classic repair" and "univentricular palliation" yield equivalent survival rates in the mid-term. Biventricular "anatomic repair", when feasible, should be promoted because of its better long-term outcome.

How well are patients doing up to 30 years after a mustard operation?

BACKGROUND: Right heart failure and baffle complications may affect the health status of patients with transposition of the great arteries after an atrial switch operation. METHODS: This study aims to identify risk factors for late death, the incidence of reoperations, and the functional status of 88 patients who underwent a Mustard operation with a mean follow-up of 20.9 +/- 10.0 years. RESULTS: There were 7 early and 19 late deaths. Follow-up was complete for 97 % of the hospital survivors. Survival and freedom from reoperation of the hospital survivors at 20 years was 83.7 +/- 4.2 %, and 70.6 +/- 5.4 %, respectively. Seven reoperations were performed for systemic ventricular failure, and 24 for baffle complications, with no operative mortality. Presence of a ventricular septal defect at the time of the Mustard operation was predictive for late death in multivariate analysis ( P = 0.040). At follow-up, 82 % of the patients were able to work full-time, 11 % part-time, and 7 % experienced noticeable limitations of their activities. CONCLUSIONS: Presence of a VSD at the time of the Mustard operation defines a distinct subgroup with an increased risk for late death. Long-term survivors were in a good functional status but had to be reoperated frequently due to baffle complications that seemed to increase in adulthood.

Surgical closure of atrial septal defect in patients older than 30 years: risk factors for late death from arrhythmia or heart failure.

BACKGROUND: Little is known about prognostic markers for late cardiac-related death after surgical atrial septal defect (ASD) closure in adults. METHODS: Long-term follow-up data of 281 patients who underwent surgical secundum ASD closure when they were older than 30 years, were retrospectively examined. RESULTS: Mean age at surgery was 43.8 +/- 10.0 years (30 to 76 years). There were 2 early deaths. Mean follow-up was 14.1 +/- 8.4 years (0.4 to 28.9 years). Death from arrhythmia or heart failure occurred in 9 patients (3.6 %) at a mean time of 8.5 +/- 6.6 years after the operation. Patients > 43 years exhibited significantly higher pulmonary artery pressures. Preoperative systolic pulmonary artery pressure > 36 mmHg, and mean pulmonary artery pressure > 21 mmHg were predictive of late death from arrhythmia or heart failure. However, age at operation was not. CONCLUSIONS: Older age at the time of ASD closure is not a risk factor for late death from arrhythmia or heart failure in adults. However, older patients presented more often with pulmonary hypertension. Since elevated pulmonary artery pressure is predictive of late death from arrhythmia or heart failure, timely ASD closure is warranted.

[Primary reconstruction of the common truncus arteriosus in infants, using modified pulmonary homograft]. / Truncus arteriosus communis primer rekonstrukciója csecsemökorban, méretre alakított pulmonalis homograft felhasználásával.

Truncus arteriosus communis (TAC) is a complex heart disease, it presents 1% of the congenital heart defects. A 7 weeks old infant with cardiac failure was operated on with TAC. The bicuspidized (14 mm diameter) pulmonary homograft valved conduit was used to reconstruct the right ventricular outflow tract (RVOT). The sternum closure was delayed. The important points of the indications for the surgical management of TAC: 1. Management or prevention of the pulmonary hypertensive crisis. 2. Patient's age. 3. Reconstruction of the RVOT (choices: homograft, dacron valve conduit, autologous pericardial valve conduit, porcine aortic root, direct anastomosis of the pulmonary trunc to the right ventricle). The surgical management is based on the optimal combination of these three important points. This is the first successfully performed primary surgical repair of an infant with TAC using modified pulmonary homograft in Hungary.

[Successful multi-step management of developmental heart defects after intrauterine diagnosis]. / Intrauterin diagnosztizált összetett szívfejlódési rendellenesség többlépcsós sikeres sebészi korrigálása.

At 28th week of gestation a conotruncal malformation with ventricular septal defect was diagnosed by fetal echocardiography. Postnatal echocardiographic and angiocardiographic examinations confirmed the diagnosis of conotruncal malformation (pulmonary atresia, ventricular septal defect, patent ductus arteriosus, aortopulmonary collateral arteries). The unifocalization (age: 11 months) and total correction with aortic homograft (age: 7 years) were performed. To our knowledge our case is the first whose intrauterine diagnosis of complex congenital heart disease was confirmed after delivery and had successful two-stage surgical management.