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Background. Extracorporeal membrane oxygenation (ECMO) has become an integral part of paediatric cardiac surgery. We report the experience of a well-established ECMO service over a 5-year period. Methods. This retrospective study analysed all paediatric patients who required ECMO support following cardiac surgery from April 2015 to March 2020. Inclusion criteria were age less than 18 and post-operative ECMO support. Patients were analysed dividing into groups according to the urgency for ECMO support (extracorporeal cardiopulmonary resuscitation (ECPR) and cardiac ECMO) and according to age (neonatal and paediatric ECMO groups). They were followed for 30-day, 6-month mortality, long-term survival, postoperative morbidity and the need for reintervention. Results. 46 patients were included who had a total of 48 ECMO runs. The 5-year incidence of the need for ECMO after cardiac surgery was 3.3% (48 of the overall 1441 cases recorded). 30-day, 6-month and follow-up survival rate was 85%, 65% and 52% respectively. At the 6-month follow-up, the ECPR group showed a trend towards worse survival compare with the cardiac ECMO group (47% vs 55%) but with no statistical significance (p = 0.35). Furthermore, the survival rates between paediatric (60%) and neonatal (46%) ECMO groups were similar, with no statistical significance (p = 0.45). The rate of acute neurological events was 27% (13/48). Conclusion. ECPR and neonatal ECMO groups had higher mortality. ECMO 30-day and 6-month survival rates were better compared with other studies 85% and 65% respectively. Incidence of ECMO and neurological complication rates were in line with other high-volume centres.

Background Pulmonary artery sling is commonly associated with tracheal stenosis and intracardiac anomalies. While surgical repair is standardized, coexistent anomalies often determine outcomes. With the paucity of risk stratification, this study aimed to review our experience and stratify risk factors for the surgical outcome of complex pulmonary artery sling repair in the presence of airway or intracardiac lesions. Methods Seventy-nine consecutive children with pulmonary artery sling were evaluated retrospectively following surgical repair. Median age at surgery was 5 months (interquartile range 3–9). Surgical approaches included pulmonary artery sling alone ( n = 10), pulmonary artery sling with tracheoplasty ( n = 41), and pulmonary artery sling with both intracardiac and tracheal surgery ( n = 28). Results There were 7 early (8.8%) deaths. Two patients after left pulmonary artery reimplantation needed revision of the anastomosis. The median intensive care and hospital stay were 11 (interquartile range 9.2–24.8) and 17.9 (interquartile range 4.3–19.8) days, and considerably longer when associated tracheal surgery ( p = 0.002). Follow-up was complete in 66/69 and 3 (3.8%) children died late: 2.7, 10.2, and 17 months after surgery. Univariate analysis showed abnormal lung and coexisting structural heart disease as risk factors. Multivariate analysis revealed total cardiopulmonary bypass time as an independent predictor of overall mortality. Conclusion Complex pulmonary artery sling repair can be performed with a good surgical outcomes even when associated with airway malformations or structural heart diseases. Lung abnormality and longer cardiopulmonary bypass time as a surrogate marker of complex surgery, are possible risk factors.

Giant congenital right atrial aneurysms are rare, with their natural history complicated by death, heart failure, arrhythmias, and thrombosis. Prenatal diagnosis of the defect is feasible and allows an early preparation of a plan for management. We present details of a patient diagnosed prenatally with a giant right atrial aneurysm, which was successfully treated surgically as soon as the patient became symptomatic.

Most single ventricle patients undergo Fontan procedure earlier in current era. However, optimal timing of Fontan completion after an intermediate staging surgery is controversial. We present children who had Fontan surgeries younger than 3 years of age comparing the data with older patients. Between 2000 and 2013, 45 patients with functional single ventricle underwent extracardiac total cavopulmonary connection (TCPC) using GORE-TEX(®) conduit at a single institution. Children were divided into group A (age 36 months; n = 15) and group B (age ≥ 36 months; n = 30), and retrospectively reviewed. Median follow-up was 2.9 [interquartile range (IQR) 2-4.4] years. There were two hospital deaths and one late death (all in group B, p = 0.286). One TCPC was taken down in group A. The median intensive care unit and hospital stay were 90 (IQR 46-140) h and 21 (IQR 16-33) days for group A versus 65 (IQR 45-115) h and 29 (IQR 20.8-38.5) days for group B, respectively (p = 0.322 and p = 0.057). Ventricular ejection fraction and GORE-TEX(®) conduit size were similar in both groups. The time of chest drain stay was significantly lower in group A (median 12 days, IQR 7-22) than in group B (22 days, IQR 16-32.5) (p = 0.014). Extracardiac TCPC can be performed earlier with good intermediate results. Earlier Fontan procedure might be advantageous for reducing chest drains stay.

An extracorporeal membrane oxygenation was long a golden standard in the pediatric population with acute cardiac failure for short-term mechanical circulatory support. It gives the limited availability of pediatric-sized pumps and the outcomes remain disproportionately poor. The Levitronix PediVAS system (Levitronix LLC, Waltham, MA) offers expanded options for short-term support for this population. We report our experience with the successful use of the PediVAS (left heart bypass) in the 3-year-old male patient as a bridge to recovery after Fontan surgery in acute heart failure. Short-term circulatory support with the Levitronix PediVAS has proven to be a less invasive, safe, and effective for our pediatric patient.

Objective: The Carpentier-Edwards (CE) Physio II ring is a new prosthetic ring designed to accommodate the changing pathology seen in the spectrum of degenerative valve disease, particularly the larger anterior leaflet in repair of the Barlow valve. The aim of our study was to assess thesafetyandefficacyofmitralvalve(MV)repairwiththeCEPhysioIIring.Methods:BetweenApril2009andMarch2010,100patientsunderwent MV repair using the Physio II ring. Median age of patients was 70 years (54—85 years). The left ventricle (LV) was moderately (30—50%; n = 21) or severely (

Tumors are rare in neonates. The most common type is a benign rhabdomyoma. Rhabdomyoma is self-regressing with time. However, if a tumor is huge and causing hemodynamic instability, surgery is indicated. We present successful cardiac surgery for partial removal of a giant rhabdomyoma tumor in the right ventricle of a 7-day-old neonate and spontaneous regression of the remnant tumor mass at follow-up.

Cześciowo nieprawidlowy splyw lewej gornej zyly plucnej wraz z ubytkiem przegrody miedzyprzedsionkowej jest rzadką wrodzoną wadą serca. Standardem w leczeniu tej kombinowanej wady serca jest operacja wykonywana poprzez sternotomie z uzyciem plucoserca. W pracy przedstawiono procedure hybrydową wykonaną u 12-letniej pacjentki podczas zastosowania jednego znieczulenia. Zabieg obejmuje przezskorne zamkniecie ubytku przegrody miedzyprzedsionkowej i korekcje gornej zyly plucnej z zastosowaniem minimalnego chirurgicznego podejścia bez uzycia plucoserca. Slowa kluczowe: wrodzone wady serca, procedura hybrydowa. Abstract

Chest wall and spine deformities are common in Marfan syndrome, and often coexist with cardiac defects. Surgery is often needed to both the aortic root and the anterior chest wall, and early spinal surgery is not uncommon. We describe a case of severe thoracic cage deformity with aortic root dilatation, which was managed by a staged approach, with a very good final result.

Background The Fontan procedure has been modified several times since it was introduced into practice in 1968. As many patients now survive to adulthood, attention is directed towards their clinical status and late morbidity. We report our surgical experience of 30 years in Fontan procedures. Methods From January 1985 to January 2015, 80 patients underwent Fontan surgery. Twenty-one patients received an atrio-pulmonary Fontan (Group I), four patients underwent total cavopulmonary connection (TCPC) with an intra-atrial lateral tunnel (Group II), six patients received extra-cardiac TCPC with an aortic homograft (group III) and 49 patients received extra-cardiac TCPC with an expanded polytetrafluoroethylene conduit. They were followed for early and late mortality, long-term survival, postoperative morbidity and reoperations. Results The mean follow-up time was 7.4 ± 6.6 years. The Kaplan–Meier estimated 15-year survival rate was 42% in Group I, 50% in Group II, 83% in Group III and 94% in Group IV. The median length of stay in intensive care unit, intubation and chest drain stay time were 90 h (IQR, 46–119), 8 h (IQR, 6–16) and 18 days (IQR, 12–28) respectively. Early complications were bleeding (6), taken down of Fontan circulation (3) and acute heart failure managed by left heart bypass (1). Late-occurring morbidities included arrhythmias (6), protein-losing enteropathy (2), thromboembolism (2) and tracheal stenosis (1). Fourteen patients (18%) had redo Fontan procedures. Conclusion Our series showed improving results after Fontan completion with excellent mid-term outcome after extra-cardiac TCPC with expanded polytetrafluoroethylene conduit. The long-term result should be followed.