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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.

Background Facing longer follow-up of patients after Fontan operation, Fontan conversion was proposed to treat failing Fontan circulation. We reviewed our patients who reached up to 42 years of age. Methods Fifteen consecutive patients underwent Fontan conversion to extracardiac conduit combined with biatrial maze procedure between October 2006 and January 2014. Mean age at conversion was 30.9 ± 5.6 years and mean interval from primary Fontan palliation to conversion was 23.2 ± 3.3 years. Results There was one early death. Ten of 14 survivors were extubated within 24 hours, and 11 were discharged from intensive care unit (ICU) within 7 days. The impaired left ventricular function, presented in four patients prior to surgery, improved from EF 43.8 to 54.8% ( p = 0.02). During follow-up time of 3.6 ± 2.3 years after conversion, there was no late death and no reoperation. Two patients developed recurrent atrial arrhythmia. Older age at Fontan procedure, lower left ventricular function, higher New York Heart Association (NYHA) class, and anatomy other than tricuspid atresia emerged as risk factors for longer ICU stay. Conclusion Fontan conversion improved the functional status in almost all patients. A concomitant maze procedure effectively eliminated atrial arrhythmia. This procedure provides a benefit even for older patients if all additional cardiac pathologies are addressed.

Purpose: To determine the results of a novel technique for interrupted aortic arch (IAA) repair in regard to survival and freedom from reintervention. With the spatulated subclavian artery a reversed flap is created to form the wall of the posterior aortic arch. Either a patch from autologous pericardium or homograft tissues are used to complete the continuity. Methods: We analyzed medical records of patients undergoing IAA repair using this technique. Results: Between April 2000 and July 2012, 27 patients have undergone IAA repair using the subclavian flap technique. Of these, 2 had an aortopulmonary window, 3 a common arterial trunk, and 3 a transposition of the great arteries. In patients with type B interruption (74%) the left subclavian artery (n = 17) or the right arteria lusoria (n = 3) were used. In type A interruption (26%) only the left subclavian artery was used as flap. Arch repair was completed using fixated autologous pericardium (41%) or a homograft patch (59%). Mean follow-up time was 4.1 ± 2.3 years. No patient was lost at late follow-up. In the early period from 2000 – 2005 (5 pts.) there was one early and one late death. Since 2006 no deaths occurred. Aortic arch stenosis requiring reintervention developed in 8 patients (30%). Four of them could be treated with balloon dilatation, four underwent surgical treatment. The rate of aortic arch stenosis was much lower in patients treated with a homograft patch versus those with a pericardial material (13% versus 55%, p = 0.038). Conclusions: The subclavian flap technique is a promising alternative to either direct anastomosis or prosthesis interposition. As a large part of the arch circumference entails use of patch material, its choice is crucial. Preliminary results raise concern about the use of autologous pericardial patch material.

Ventricular septal defects (VSDs) are a common congenital heart disease. Usually, surgical repair with cardiopulmonary bypass (CPB) is the treatment of choice, whereas percutaneous techniques have technical limitations, predominantly a mismatch of catheter size and body weight. A 7-year-old girl underwent periventricular closure of a perimembranous VSD on the beating heart. Echocardiography guided implantation through a minimally invasive sternotomy was uneventful. The described approach adds favorably to the current practice avoiding the use of CPB. Cosmetic aspect and rapid early postoperative recovery are convincing.

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.

Background e-Aminocaproic acid (EACA) and tranexamic acid (TXA) are used for antifibrinolytic therapy in neonates undergoing cardiac surgery, although data directly comparing their blood-sparing efficacy are not yet available. We compared two consecutive cohorts of neonates for the effect of these two medications on perioperative blood loss and allogeneic transfusions. Material and methods Data from the EACA group (n = 77) were collected over a 12-month period; data from the tranexamic acid group (n = 28) were collected over a 5-month period. Blood loss, rate of reoperation due to bleeding, and transfusion requirements were measured. Results There was no significant difference in blood loss at 6 hours (EACA 24 [17-30] mL/kg [median (interquartile range)] vs. TXA 20 [11-34] mL/kg, P = 0.491), at 12 hours (EACA 31 [22-38] mL/kg vs. TXA 27 [19-43] ml/kg, P = 0.496) or at 24 hours postoperatively (EACA 41 [31-47] mL/kg vs. TXA 39 [27-60] mL/kg; P = 0.625) or transfusion of blood products. Conclusions e-Aminocaproic acid and tranexamic acid are equally effective with respect to perioperative blood loss and transfusion requirements in newborns undergoing cardiac surgery.

BACKGROUND: Pulmonary atresia with intact ventricular septum (PA-IVS) is a complex congenital heart defect with a large variety of right heart-sided morphologies. METHODS: We undertook a retrospective review of 86 patients with PA-IVS with a special emphasis on the angiographic findings. The aim of the study was to determine predictors for biventricular repair. Initial surgical procedures depended on the right ventricular morphology, the tricuspid valve size and coronary anomalies. RESULTS: Fifty-five patients (64 %) underwent decompression of the right ventricle (RV) as an initial procedure; 16 of them required an additional systemic-to-pulmonary artery shunt. Twenty-six patients (30 %) had only a systemic-to-pulmonary artery shunt as their initial procedure. Five patients underwent interventional procedures performed by pediatric cardiologists. Biventricular repair was possible in 56 patients (65 %). Univentricular palliation was achieved in 16 patients. Fourteen patients had only palliation with a systemic-to-pulmonary artery shunt. Mean tricuspid valve size was significantly bigger in patients with biventricular repair (z-score −3.6 ± 2.6) than in patients who did not undergo biventricular repair (−5.2 ± 1.7, P = 0.003). Predictors for biventricular repair were right ventricular decompression with or without systemic-to-pulmonary artery shunt ( P < 0.001), tripartite right ventricle ( P < 0.001) and the absence of coronary fistulae ( P < 0.001). Long-term survival was 80 % ± 13 % at 25 years for patients undergoing biventricular repair. CONCLUSIONS: Decompression of the RV as an initial surgical procedure improves the possibility of achieving biventricular repair with good long-term results. However, morphological factors such as right ventricular size and the absence of coronary fistulae are significant predictors for biventricular repair.

Objective: An increasing number of surgeons prefer to place an right ventricle to pulmonary artery (RV-PA) conduit at the time of Norwood stage I. Proximal shunt stenoses have led us to modify this technique using ring-enforced polytetrafluroethylene shunts. Methods: Angiograms of 22 patients with conventional shunts (CS) and 18 patients with ring-enforced shunts (RS) prior to bidirectional cavopulmonary anastomosis were analyzed. Diameter of any shunt was 5mm. Results: In the RS group the extent of a shunt stenosis at the level of the proximal anastomosis, and within the substernal proximal third of the shunt was minimized (30±25% vs. 47±21%, p=0.023, and 8±7% vs. 47±26%, p

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.

Objectives: Pregnancy in women with a heart valve prosthesis, might lead to a faster deterioration of a biological prosthesis or embryopathy due to anticoagulation. We sought to look at the outcome of pregnancies in patients with a valve prosthesis. Methods: At time of the study, 165 female patients who had had a valve replacement between 1976–2006, were 18–40 years old. Evaluation of the outcome of pregnancies and their course as well as reoperations was undertaken. Results: Questionnaires (n=136) were evaluated at a mean time of 9.72±7 years after valve replacement. A mechanical prosthesis was implanted in 49 patients (36%), 41 patients (30%) had a homograft placement and 46 patients (34%) received a biological prosthesis. After valve replacement, 42 patients (30.9%) had a total of 70 pregnancies with a live birth rate of 65.7% (n=46). The rate of pregnancy was higher for patients with a biological prosthesis (n=20, 43.5% p=0.04) or a homograft (n=14, 34.1%, p=0.03) than for patients with a mechanical prosthesis (n=8, 16.3%). The rate of abortion was higher with a mechanical prosthesis (n=5, p=0.001), two were indicated for neonatal malformation. There were no differences between the rate of stillbirths and miscarriages. Degeneration of the biological valve prosthesis was not accelerated by the pregnancy. Conclusion: Patients with a mechanical prosthesis are at higher risk of having an unsuccessful pregnancy, compared to patients with a biological or homograft valve. Young women wishing to conceive and requiring a valve replacement should be advised to opt for a biological prosthesis.

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.