Heart Transplantation after Univentricular Palliation: Improved Outcomes and Increased Complexity.

AIM: Heart transplantation (HT) in patients with failing univentricular circulation is often challenging. This is compounded by the ever-increasing number of patients with prior Norwood-type reconstruction of the aorta, large aortic root, and often dense adhesions from multiple prior operations. We aimed to elucidate differences in outcomes of HT in patients with prior univentricular palliations, with and without prior Norwood-type aortic arch reconstruction (ArchRec). METHODS: All patients who underwent HT for failed univentricular palliation during the 1990-2022 period were included in the study. RESULTS: Of 45 patients, 18 had undergone ArchRec. Hospital mortality improved in the recent era (17.4% before 2006 vs 0% after 2006; p=0.11), despite a higher proportion of patients with ArchRec (17.4% before 2006 vs 60.8% after 2006, p=0.002). Patients with ArchRec had a higher number of prior cardiac surgeries (4.1±1.5 vs 3.2±1.3, p=0.04), longer cardiopulmonary bypass time (320±23 vs 242±21 min, p=0.02), more concomitant arch reconstruction (33.3% vs 0%, p=0.02), greater need for post-HT extracorporeal membrane oxygenation (33.3% vs 3.7%; p=0.01) and longer hospital stay (37.1±30.5 days vs 23.6±11.8 days, p=0.04). Freedom from death or retransplantation for all patients was 91%, 73%, 67%, and 53% at 1, 5, 10, and 15-years, respectively. Prior ArchRec, Fontan procedure, and earlier eras were not risk factors for death. CONCLUSIONS: The outcomes of HT after univentricular palliation have improved in recent times and low operative mortality can be achieved. Despite increased complexity, good similar outcomes can be achieved in patients with and without prior arch reconstruction regardless of the palliation stage.

Arterial switch operation in a child with commissural malalignment and unusual coronary anatomy.

The arterial switch operation has evolved to become the treatment of choice for transposition of the great arteries and is one of the greatest success stories in congenital heart surgery. The most crucial step of the operation is the coronary artery translocation; therefore, it is of paramount importance for surgeons to know every single detail about the morphology and spatial relationships of the coronary arteries and the roots of the great vessels. However, sometimes the surgeon may face unfavourable scenarios such as major commissural malalignment and anomalous coronary artery patterns and need to be prepared to carry out a successful coronary artery translocation. Herein, we demonstrate that the trapdoor technique is useful for transferring coronary arteries in a neonate with major commissural malalignment and unusual coronary anatomy during the arterial switch operation.

Truncal valve repair in an adolescent with severe annular dilatation.

Approximately 20% of patients with truncus arteriosus might need a truncal valve procedure within 20 years after anatomical repair due to regurgitation. These patients commonly develop valve regurgitation due to root dilatation with a sufficient amount of good quality valvular tissue. Thus, the reduction of the truncal annulus is the single most important factor to achieve durable repair, especially in patients in whom the Ross procedure is not an option.

Aortic valve repair in a low-birth-weight neonate with poor ventricular function: staged tricuspidization of the unicuspid valve.

Congenital severe isolated aortic stenosis in neonates requires prompt intervention because left ventricular function may deteriorate rapidly. Surgical repair of a stenotic aortic valve in neonates allows debridement of fibrous material and precision commissurotomy. As such, the child would end up with a much better-quality aortic valve compared to the uncontrolled tear that results following balloon dilatation. The meticulous debridement and precise commissurotomy are important in achieving long-term durability of the aortic valve repair, with the expectation that the patient may keep the native aortic valve at least into adolescence or adulthood. If feasible, it is important to avoid placement of patches while repairing the valve, so that the growth potential of the cusps is preserved. The valve could be revisited and repaired further, so that, ideally, the native valve could be retained for life, thus avoiding aortic valve replacement entirely. We present a low-birth-weight neonate with a dysplastic unicuspid aortic valve, severe aortic valve stenosis and decreased left ventricular function who underwent repair of the aortic valve.

Pulmonary valve replacement for treatment of severe pulmonary regurgitation in patients with previous tetralogy of Fallot repair surgery.

Tetralogy of Fallot was classically described as the combination of pulmonary stenosis, right ventricle hypertrophy, perimembranous ventricular septal defect and an aortic root that overrides the ventricular septal defect. Full surgical repair, which is usually indicated in toddlers, comprises closure of the ventricular septal defect and alleviation of the pulmonary stenosis. Because there is a wide anatomical spectrum, the cause and severity of the pulmonary stenosis are variable. However, when the pulmonary valve is stenotic and not well-developed, it has to be removed, and one has to accept severe pulmonary regurgitation as a sequela. In the ensuing years, when signs of damage to the right ventricle are proven, pulmonary valve replacement is indicated. We present the case of a 16-year-old patient with tetralogy of Fallot that was corrected during the first year of life. Follow-up demonstrated progressive exercise intolerance and moderate-to-severe right ventricle dilatation. Thus, surgical pulmonary valve replacement was indicated. The surgical approach was through a full median redo sternotomy. Central cardiopulmonary bypass was established with bicaval drainage. The operation was performed under assisted non-clamped circulatory support. The calcified previous pericardial patch was removed, and a 25-mm bioprosthetic valve was implanted in the pulmonary annulus. A new pericardial heterologous patch was used for the transannular pulmonary plasty.

Oxidative Stress and Indicators of Brain Damage Following Pediatric Heart Surgery.

Pediatric cardiac surgery induces an increased oxidative stress (OS) response. Increased OS is associated with poor neurologic outcomes in neonatal populations with similar patterns of brain injury. We investigated OS and brain injury in infants undergoing heart surgery. Patients 6 months or younger, undergoing cardiac surgery with or without cardiopulmonary bypass (CPB), were included in this prospective, observational study. Patients were divided into infant (30 days−6 months) and neonatal (<30 days) groups for analysis. Urine OS biomarker 8-iso-prostaglandin F2α (8-iso-PGF2α) was quantified pre-surgery and at 0 and 24 h post-surgery. A serum brain damage biomarker S100B protein was also measured pre-surgery and at 0 and 72 h post-surgery. Amplitude-integrated electroencephalography during surgery was analyzed. Neuropsychological evaluation using the Bayley III or Vineland test was performed in all patients at 24 months of age. Sixty-two patients were included, 44 of whom underwent follow-up neurologic evaluation. 8-iso-PGF2α and S100B levels were increased after surgery. Postoperative levels of S100B were positively correlated with 8-iso-PGF2α levels 24 h after surgery (rho = 0.5224; p = 0.0261). There was also a correlation between immediate post-surgery levels of 8-iso-PGF2α and intra-surgery seizure burden (rho = 0.4285, p = 0.0205). Patients with an abnormal neurological evaluation had increased levels of S100B 72 h after surgery (p = 0.048). 8-iso-PGF2α levels 24 h after surgery were also related to abnormal neurologic outcomes. Levels of 8-iso-PGF2α following pediatric cardiac surgery are associated with several indicators of brain injury including brain damage biomarkers, intra-operative seizures, and abnormal neurological evaluation at follow-up, suggesting the importance of oxidative stress response in the origin of brain damage in this population.

A Rare Case of Cardiogenic Shock: Cor Triatriatum With Anomalous Pulmonary Venous Return.

Obstructive total anomalous pulmonary venous return (TAPVR) is a potentially life-threatening clinical condition and a surgical emergency. Left-sided partial anomalous pulmonary venous return (PAPVR) in association with a subdivided left atrium, also known as cor triatriatum sinister (CTS), is a very rare malformation that can mimic obstructive TAPVR. We present a case of a newborn with clinical manifestation of cardiogenic shock caused by CTS and PAPVR.

Biventricular fibroma resection in an adult.

Cardiac fibromas are the second most common primary cardiac tumors in childhood. They only rarely present in adults. They are mainly located in the free wall of the ventricles and attached to the interventricular septum. Despite their benign pathology, fibromas in some locations can cause significant complications. This video tutorial illustrates the resection of a septo-apical cardiac fibroma in an asymptomatic 26-year-old woman, diagnosed with transthoracic echocardiography and cardiac magnetic resonance imaging after an abnormal ECG on an occupational medical examination. Surgery was performed through a median sternotomy using cardiopulmonary bypass. A nodular intramyocardial mass was found at the left ventricular apex and resection was carried out. After removing the fibroma, a defect was found at the apex affecting both ventricles. The defects were closed using a direct suture of 4-0 polypropylene "U" stitches reinforced with a Teflon patch to exclude the ventricular cavity.  Pathology confirmed the diagnosis of benign cardiac fibroma with tumor-free surgical margins. The patient recovered uneventfully and was discharged on postoperative day 5. The outpatient follow-up cMRI 10 months after surgery showed no signs of tumor recurrence and normal biventricular function.

Surgical rescue after left anterior descending catheter entrapment causing cardiogenic shock.

Entrapment or device loss during percutaneous coronary intervention is a rare but potentially fatal complication. Percutaneous retrieval is possible but surgery can be required on an emergency basis. We present the case of an entrapped balloon catheter in the left anterior descending coronary artery during an elective percutaneous coronary intervention. The patient developed acute myocardial ischaemia and cardiac arrest. Emergency surgical intervention with device retrieval and distal bypass grafting was life-saving.