Becoming a congenital heart surgeon: the long and challenging road.

Training in congenital cardiac surgery is potentially lengthier and more demanding than training in any other surgical field. The duration of training is proportional to the complexity of the specialization. The expertise of a wide range of procedures is required. There is no doubt that some individuals may acquire the requisite abilities with greater ease than others, but fundamentally, these are capabilities that can be taught and learnt. Moreover, congenital cardiac surgeons are required to have a detailed understanding of pathophysiology and morphology, in addition to the stamina and empathy required to manage these complex patients. A fellowship is just the start of such training and is followed by a long road eventually leading to a lifelong journey to become a qualified congenital cardiac surgeon. Effective mentorship is a prerequisite throughout training to guide surgeons on this journey.

Risk evaluation in adult congenital heart surgery: analysis of the Society of Thoracic Surgeons Congenital Heart Surgery Database risk models on data from the European Congenital Heart Surgeons Association Congenital Database.

OBJECTIVES: We sought to evaluate the predictive power of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database. METHODS: The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database. Each operation was assigned a STAT mortality score and category and an ACHS mortality score. Operative mortality was based on the 30-day status and on the status at hospital discharge. The discriminatory power of the STAT and ACHS scores was assessed using the area under the receiver operating characteristic curve (c-index). RESULTS: A total of 17 214 (97.46%) operations were assigned ACHS scores. The 3 most frequent primary procedures were closure of the atrial septal defect (19.0%), aortic valve replacement (8.8%) and non-valve-sparing aortic root replacement (6.1%). Operative mortality for ACHS-coded operations was 2.07%. The procedures with the highest mortality were atrial septal defect creation/enlargement (19.0%), lung transplantation (18.8%) and heart transplantation (18.2%). A total of 17 638 (99.86%) operations were assigned a STAT score and category. The operative mortality for STAT-coded operations was 2.27%. The c-index for mortality was 0.720 for the STAT mortality score and 0.701 for the ACHS score. CONCLUSIONS: The ACHS mortality score and the STAT mortality score reached similar, moderate predictive power in adult patients undergoing congenital heart surgery in ECHSA database.

Replacement of aprotinin by ε-aminocaproic acid in infants undergoing cardiac surgery: consequences for blood loss and outcome.

BACKGROUND: Once aprotinin was no longer available for clinical use, ε-aminocaproic acid (EACA) and tranexamic acid became the only two options for antifibrinolytic therapy. We compared aprotinin and EACA with respect to their blood-sparing efficacy and other major clinical outcome criteria in infants undergoing cardiac surgery. METHODS: We retrospectively analysed data from a large consecutive cohort of infants (n=227) aged 31-365 days undergoing primary cardiac surgery requiring cardiopulmonary bypass encompassing the transition from aprotinin to EACA (aprotinin n=88, EACA n=139); all other aspects including the medical team and departmental protocols remained unchanged. The primary outcome was postoperative blood loss measured as chest tube output (CTO). Secondary outcome parameters were transfusion requirements, reoperation due to bleeding, renal, vascular, and neurological complications, and in-hospital mortality. RESULTS: CTO was significantly higher in the EACA patients {aprotinin 18 (13-27) ml kg(-1) 24 h(-1), EACA 23 (15-37) ml kg(-1) 24 h(-1) [mean (inter-quartile range)], P=0.001}, but transfusion requirements and donor exposures were not significantly different. A sensitivity analysis strengthened our finding that the increased blood loss in the EACA group was attributable to lower efficacy of EACA. There were no significant differences in the other clinical outcome measures. CONCLUSIONS: CTO was lower in aprotinin-treated patients. Nonetheless, EACA remains a suitable substitute without measurable differences in other clinical outcome criteria.

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.

Comparison of blood-sparing efficacy of ε-aminocaproic acid and tranexamic acid in newborns undergoing cardiac surgery.

BACKGROUND: ε-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: ε-Aminocaproic acid and tranexamic acid are equally effective with respect to perioperative blood loss and transfusion requirements in newborns undergoing cardiac surgery.

Predictors for biventricular repair in pulmonary atresia with intact ventricular septum.

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.

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.

A comparative study of rat and human Tmp21 (p23) reveals the pseudogene-like features of human Tmp21-II.

Tmp21 (p23) is involved in biosynthetic transport from the endoplasmic reticulum to the Golgi complex. We have recently characterized two cDNA-variants of human Tmp21, the Tmp21-isoforms-I and -II. Because of the lack of cDNA sequence data and protein expression data, it was not clear if Tmp21-II encodes a functional Tmp21-protein. Here we describe the cloning of the full length human Tmp21-II transcript. The putative open reading frame of Tmp21-II contains a reading frame jump and a nonsense mutation in comparison to all other Tmp21-I (p23) members. Our data indicate that hum-Tmp21-II is transcribed, but not translated. We conclude that Tmp21-II cDNA derives from a neutral pseudogene, which originates from a duplication event of the human Tmp21-isoform-I.