Computed tomographic-based three-dimensional printing of giant coronary artery fistulas to guide surgical strategy: a case series.

Background: Coronary artery fistulas (CAFs) are abnormal communications between the coronary arteries and the heart chambers, arteries, or veins, potentially leading to significant shunting, myocardial ischaemia and heart failure. Computed tomographic (CT) angiography or conventional invasive angiography is the reference standard for the diagnosis of coronary fistulas. The fistula anatomy can become very complex, which makes surgical or interventional planning challenging. Case summary: We report two cases of hugely dilated and tortuous coronary circumflex artery fistulas draining into the coronary sinus. Both patients were followed up for more than 10 years because of very complex coronary fistula anatomy and mild symptoms. From two-dimensional (2D) sliced CT images alone it, was uncertain whether surgery was feasible. However, since both patients had symptom progression (Patient 1 developed heart failure, and Patient 2 had recurrent pericardial effusions), three-dimensional (3D) heart models were printed for better understanding of the complex fistula anatomy and improved surgical planning. Both patients had successful surgery and symptomatic relief at follow-up. Discussion: The delay in surgery, until clinical deterioration, may partly be a consequence of a general reluctance in performing complex surgery in patients with CAFs. As of now, CT-based 3D printing has primarily been used in isolated cases. However, 3D printing is evolving rapidly and supplementing 2D sliced CT images with a physical 3D heart model may improve the anatomical understanding and pre-surgical planning that could lead to better surgical outcome.

Pulmonary Valve Replacement in Tetralogy of Fallot: Procedural Volume and Durability of Bioprosthetic Pulmonary Valves.

BACKGROUND: Robust data on changes in pulmonary valve replacement (PVR) procedural volume and predictors of bioprosthetic pulmonary valve (BPV) durability in patients with tetralogy of Fallot (TOF) are scarce. OBJECTIVES: This study sought to assess temporal trends in PVR procedural volume and BPV durability in a nationwide, retrospective TOF cohort. METHODS: Data were obtained from patient records. Robust linear regression was used to assess temporal trends in PVR procedural volume. Piecewise exponential additive mixed models were used to estimate BPV durability, defined as the time from implantation to redo PVR with death as a competing risk, and to assess risk factors for reduced durability. RESULTS: In total, 546 PVR were performed in 384 patients from 1976 to 2021. The annual number of PVR increased from 0.4 to 6.0 per million population (P < 0.001). In the last decade, the transcatheter PVR volume increased by 20% annually (P < 0.001), whereas the surgical PVR volume did not change significantly. The median BPV durability was 17 years (Q1: 10-Q3: 10 years-not applicable). There was no significant difference in the durability of different BPV after adjustment for confounders. Age at PVR (HR: 0.78 per 10 years from <1 year; 95% CI: 0.63-0.96; P = 0.02) and true inner valve diameter (9-17 mm vs 18-22 mm HR: 0.40; 95% CI: 0.22-0.73; P = 0.003 and 18-22 mm vs 23-30 mm HR: 0.59; 95% CI: 0.25-1.39; P = 0.23) were associated with reduced BPV durability in multivariate models. CONCLUSIONS: The PVR procedural volume has increased over time, with a greater increment in transcatheter than surgical PVR during the last decade. Younger patient age at PVR and a smaller true inner valve diameter predicted reduced BPV durability.

Temporal changes in the surgical management of patients with tetralogy of Fallot in Denmark: a nationwide cohort study.

OBJECTIVES: To assess temporal changes in the surgical management of patients with tetralogy of Fallot including the timing of interventions, surgical techniques, reinterventions and survival in a nationwide cohort. METHODS: Patients with tetralogy of Fallot in Denmark were divided into 3 eras based on their year of birth: early (1977-1991), intermediate (1992-2006) and late (2007-2021). RESULTS: The cohort consisted of 745 patients. Median follow-up was 21.2 years (13.7-30.5). There was a temporal trend towards less shunt palliation (-0.3% per year, 95% CI -0.05 to -0.1). Median age at intracardiac repair was 2.9 years (1.8-5.0), 0.8 years (0.5-1.3) and 0.5 years (0.4-0.7) (P < 0.001) in the early, intermediate and late era, respectively. There was a temporal trend towards less valve-sparing repair (-0.7% per year, 95% CI -0.5 to -1.0) and more repair with transannular patches (0.7% per year, 95% CI 0.5-1.0). Survival at 10 years was 79% (64-76), 90% (87-93) and 95% (92-98) (P < 0.001) and pulmonary valve replacement within the first 10 years after intracardiac repair was performed in 3% (1-6), 12% (8-16) and 21% (13-29) (P < 0.001) in the early, intermediate and late era, respectively. CONCLUSIONS: There was a temporal trend towards less shunt palliation and intracardiac repair at a younger age with more use of transannular patches. While survival throughout childhood and adolescence has improved, more patients undergo pulmonary valve replacement during the first 10 years after intracardiac repair.

[Hybrid treatment of protein-losing enteropathy in a patient with univentricular circulation].

Protein-losing enteropathy (PLE) is a severe Fontan complication. This is a case report of the first hybrid treatment of PLE in Denmark of an 11-year-old Fontan patient with severe symptoms (diarrhoea, fatigue and swelling) and low albumin level. Diagnostics included intranodal and intrahepatic dynamic contrast magnetic resonance lymphangiography. The hybrid intervention consisted of selective lymphatic duct embolisation and innominate vein turn-down to treat PLE. The interventions went well, and two months after discharge the patient was relieved from PLE symptoms, the albumin level was normalised, and the patient felt more energetic.

Total Anomalous Pulmonary Venous Connection in Mother and Son with a Central 22q11.2 Microdeletion.

In this clinical report, we describe a male infant and his mother, who had similar congenital heart defects. They were both diagnosed neonatally with total anomalous pulmonary venous connection (TAPVC) in combination with other heart defects. Neither of the two had any other organ malformations or dysmorphic facial features. SNP-array identified a central 22q11.2 microdeletion in the male infant and his mother as well as in the maternal grandmother and maternal aunt. The mother and the maternal aunt additionally harbored a 15q11.2 BP1-BP2 microdeletion. The maternal grandmother was unaffected by heart disease. However, heart computed tomography scan of the maternal aunt revealed a quadricuspid aortic valve. Additionally, the maternal grandmother and the maternal aunt both had significant learning disabilities. Rarely, TAPVC has been described in patients with the common 22q11.2 microdeletions. However, to the best of our knowledge, TAPVC has not previously been reported in patients with this small central 22q11.2 microdeletion. Haploinsufficiency of TBX1 was originally thought to be the main cause of the 22q11.2 microdeletion syndrome phenotype, but TBX1 is not included in the atypical central 22q11.2 microdeletion. Previous reports have suggested an association between TAPVC and the 15q11.2 BP1-BP2 microdeletion. Our report does not support this association as the maternal aunt, who harbors both microdeletions, is unaffected by TAPVC, and the male infant affected by TAPVC does not harbor the 15q11.2 BP1-BP2 microdeletion. Our findings support that genes located in the central 22q11.2 region are important for heart development and that haploinsufficiency of these genes plays a crucial role in the development of the rare heart defect TAPVC.

Current Status of Training and Certification for Congenital Heart Surgery Around the World: Proceedings of the Meetings of the Global Council on Education for Congenital Heart Surgery of the World Society for Pediatric and Congenital Heart Surgery.

The optimal training of the highly specialized congenital heart surgeon is a long and complex process, which is a significant challenge in most parts of the world. The World Society for Pediatric and Congenital Heart Surgery (WSPCHS) has established the Global Council on Education for Congenital Heart Surgery as a nonprofit organization with the goal of assessing current training and certification and ultimately establishing standardized criteria for the training, evaluation, and certification of congenital heart surgeons around the world. The Global Council and the WSPCHS have reviewed the present status of training and certification for congenital cardiac surgery around the world. There is currently lack of consensus and standardized criteria for training in congenital heart surgery, with significant disparity between continents and countries. This represents significant obstacles to international job mobility of competent congenital heart surgeons and to the efforts to improve the quality of care for patients with Congenital Heart Disease worldwide. The purpose of this article is to summarize and document the present state of training and certification in congenital heart surgery around the world.

Infective endocarditis in right ventricular outflow tract conduits: a register-based comparison of homografts, Contegra grafts and Melody transcatheter valves.

OBJECTIVES: The aim was to investigate the incidence of infective endocarditis (IE) in right ventricle-to-pulmonary artery conduits implanted at a Danish tertiary centre. METHODS: Cases of IE in patients with homografts, Contegra grafts and Melody transcatheter valves were evaluated retrospectively with regard to the likeliness of the diagnosis using the modified Duke criteria and the likeliness of conduit involvement. Incidence rates for IE were calculated 1 and 5 years after valve implantation for all 3 conduits, and separately for Melody subgroups depending on which conduit served as landing zone. Cox regression with time-dependent covariates was used to model the impact of the conduit type on the incidence of IE. RESULTS: Annualized incidence rates of IE in homografts, Contegra grafts and Melody valves were 0.40% (0.40 cases per 100 patient-years), 0.97% and 6.96% 1 year and 0.27%, 1.12% and 2.89% 5 years after valve implantation. Hazard ratios (HRs) were 3.20 [95% confidence interval (CI) 0.91-11.17, P = 0.069] for Contegra grafts and 11.89 (95% CI 2.91-48.48, P < 0.001) for Melody valves compared to homografts. CONCLUSIONS: Bovine pulmonary conduits were more prone to endocarditis, with Melody valves being the most frequently infected. HRs for the risk of suffering from endocarditis were substantially higher for Melody valves and Contegra grafts compared to homografts, although this finding was only statistically significant for Melody valves and not for Contegra grafts.

[Mediastinal cystic component as cause of deviating growth in a ten-month-old boy].

We present a case report of a ten-month-old boy, who was referred due to deviating growth and recurrent respiratory tract infections. Computed tomography of the thorax showed two large, cystic components in the mediastinum. He underwent surgical removal of the cysts. Pathological examination confirmed the diagnosis lymphatic malformation. This case illustrates that intrathoracic tumors such as lymphatic malformations, although rare, should be considered in children with deviating growth curves and respiratory problems. Rather than continuous symptomatic treatment children with symptoms early in life should be prompted in further investigations.

Surgical options after Fontan failure.

OBJECTIVE: The objective of this European multicenter study was to report surgical outcomes of Fontan takedown, Fontan conversion and heart transplantation (HTX) for failing Fontan patients in terms of all-cause mortality and (re-)HTX. METHODS: A retrospective international study was conducted by the European Congenital Heart Surgeons Association among 22 member centres. Outcome of surgery to address failing Fontan was collected in 225 patients among which were patients with Fontan takedown (n=38; 17%), Fontan conversion (n=137; 61%) or HTX (n=50; 22%). RESULTS: The most prevalent indication for failing Fontan surgery was arrhythmia (43.6%), but indications differed across the surgical groups (p<0.001). Fontan takedown was mostly performed in the early postoperative phase after Fontan completion, while Fontan conversion and HTX were mainly treatment options for late failure. Early (30 days) mortality was high for Fontan takedown (ie, 26%). Median follow-up was 5.9 years (range 0-23.7 years). The combined end point mortality/HTX was reached in 44.7% of the Fontan takedown patients, in 26.3% of the Fontan conversion patients and in 34.0% of the HTX patients, respectively (log rank p=0.08). Survival analysis showed no difference between Fontan conversion and HTX (p=0.13), but their ventricular function differed significantly. In patients who underwent Fontan conversion or HTX ventricular systolic dysfunction appeared to be the strongest predictor of mortality or (re-)HTX. Patients with valveless atriopulmonary connection (APC) take more advantage of Fontan conversion than patients with a valve-containing APC (p=0.04). CONCLUSIONS: Takedown surgery for failing Fontan is mostly performed in the early postoperative phase, with a high risk of mortality. There is no difference in survival after Fontan conversion or HTX.

Urinary apolipoprotein M as a biomarker of acute kidney injury in children undergoing heart surgery.

AIM: To investigate whether apoM is excreted in urine of children undergoing heart surgery and the potential of apoM as early biomarker of acute kidney injury (AKI). MATERIALS & METHODS: Urine was collected in children undergoing heart surgery. ApoM was measured with ELISA. U-apoM was characterized by gel filtration chromatography and western blotting. RESULTS: ApoM was excreted into the urine 0-4 h postoperatively as the full-length apoM in particles smaller than plasma HDL. At 0 h, U-apoM predicted AKI with an area under the receiver-operating characteristics curve of 0.70 (p < 0.018). Sensitivity was 0.71 and specificity was 0.68 at a cutoff level at 1.45 nmol/l. CONCLUSION: ApoM is excreted in the urine of children after cardiac surgery. Its potential as biomarker of AKI deserves exploration.

[Pulse oximetry screening of newborns can prevent circulatory collapse caused by congenital heart defect]. / Screening af nyfødte med pulsoximetri kan hindre kredsløbskollaps forårsaget af medfødt hjertesygdom.

We present a case of total anomalous pulmonary venous drainage. Despite low oxygen saturation an eight-week-old girl had only minimal symptoms initially. She suffered collapse requiring acute surgical correction and prolonged intensive care. Her collapse and complicated post-operative course could have been avoided with earlier diagnosis. Infants with critical heart disease continue to be born undiagnosed despite prenatal ultrasound screening. There is evidence that infants with critical congenital heart defect can be detected by pulse oximetry screening, as is routine in Norway, Sweden and Finland, but not in Denmark.

Diagnosis and prognosis of anomalous origin of the left coronary artery from the pulmonary artery.

INTRODUCTION: Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is an uncommon congenital heart abnormality. The aim of this study was to describe a single-centre experience with surgical repair of this condition. METHODS: We performed a retrospective analysis of cases from February 2004 to January 2014. RESULTS: Ten patients presented with the diagnosis of ALCAPA. A total of seven infants and three adults underwent surgical repair in our Department of Thoracic Surgery, Rigshospitalet, Denmark. The seven infants presented with symptoms of heart failure: dyspnoea, sweating or failure to thrive; two adults were asymptomatic and one adult presented with cardiac arrest. Six infants had moderate to severe mitral valve regurgitation and five of these patients had preoperative moderate to severely reduced left ventricular function. Nine patients underwent surgical repair by re-implantation of the left coronary artery to the aorta and one underwent surgical repair ad modus Takeuchi (an aortopulmonary window). None of the patients underwent re-operation and none died. CONCLUSION: All ten patients survived with recovery of left ventricular function within 12 months. An early diagnosis and prompt surgical intervention is warranted in the treatment of ALCAPA.

[Pulse oximetry screening of newborns can prevent circulatory collapse caused by congenital heart defect]. / Screening af nyfødte med pulsoximetri kan hindre kredsløbskollaps forårsaget af medfødt hjertesygdom.

We present a case of total anomalous pulmonary venous drainage. Despite low oxygen saturation an eight-week-old girl had only minimal symptoms initially. She suffered collapse requiring acute surgical correction and prolonged intensive care. Her collapse and complicated post-operative course could have been avoided with earlier diagnosis. Infants with critical heart disease continue to be born undiagnosed despite prenatal ultrasound screening. There is evidence that infants with critical congenital heart defect can be detected by pulse oximetry screening, as is routine in Norway, Sweden and Finland, but not in Denmark.

[In-utero treatment of hypoplastic left heart syndrome]. / Intrauterin behandling af hypoplastisk venstre ventrikel-syndrom.

In-utero treatment of fetal aortic stenosis (AS) may prevent hypoplastic left heart syndrome. A girl was diagnosed prenatally with severe AS and was referred to the Women's and Children's Hospital in Linz, Austria, where she underwent an intrauterine valvuloplasty of the aortic valve. Postnatally, the girl was given prostaglandin and operated a.m. Ross-Konno. An echocardiography at the age of four months showed a neoaorta without stenosis and insufficiency and a normal systolic function of the left ventricle. This treatment must be carefully considered when dealing with fetuses with AS.

Favourable mid-term outcome after heart transplantation for late Fontan failure.

OBJECTIVES: Fontan failure (FF) represents a growing and challenging indication for paediatric orthotopic heart transplantation (OHT). The aim of this study was to identify predictors of the best mid-term outcome in OHT after FF. METHODS: Twenty-year multi-institutional retrospective analysis on OHT for FF. RESULTS: Between 1991 and 2011, 61 patients, mean age 15.0 ± 9.7 years, underwent OHT for failing atriopulmonary connection (17 patients = 27.8%) or total cavopulmonary connection (44 patients = 72.2%). Modality of FF included arrhythmia (14.8%), complex obstructions in the Fontan circuit (16.4%), protein-losing enteropathy (PLE) (22.9%), impaired ventricular function (31.1%) or a combination of the above (14.8%). The mean time interval between Fontan completion and OHT was 10.7 ± 6.6 years. Early FF occurred in 18%, requiring OHT 0.8 ± 0.5 years after Fontan. The hospital mortality rate was 18.3%, mainly secondary to infection (36.4%) and graft failure (27.3%). The mean follow-up was 66.8 ± 54.2 months. The overall Kaplan-Meier survival estimate was 81.9 ± 1.8% at 1 year, 73 ± 2.7% at 5 years and 56.8 ± 4.3% at 10 years. The Kaplan-Meier 5-year survival estimate was 82.3 ± 5.9% in late FF and 32.7 ± 15.0% in early FF (P = 0.0007). Late FF with poor ventricular function exhibited a 91.5 ± 5.8% 5-year OHT survival. PLE was cured in 77.7% of hospital survivors, but the 5-year Kaplan-Meier survival estimate in PLE was 46.3 ± 14.4 vs 84.3 ± 5.5% in non-PLE (P = 0.0147). Cox proportional hazards identified early FF (P = 0.0005), complex Fontan pathway obstruction (P = 0.0043) and PLE (P = 0.0033) as independent predictors of 5-year mortality. CONCLUSIONS: OHT is an excellent surgical option for late FF with impaired ventricular function. Protein dispersion improves with OHT, but PLE negatively affects the mid-term OHT outcome, mainly for early infective complications.

Potts shunt in a child with end-stage pulmonary hypertension after late repair of ventricular septal defect.

We report on a 10-year-old boy with medically refractory pulmonary arterial hypertension (PAH) and end-stage right heart failure after closure of a ventricular septal defect. The boy was a candidate for lung transplantation (LTX), but an alternative option was to create an Eisenmenger physiology with right-to-left shunting. The shunt could be created either as an intracardiac or as an extracardiac shunt. We decided to create a Potts shunt, a direct anastomosis between the left pulmonary artery and the descending aorta. The Potts shunt functioned as a right-to-left shunt, thus reducing the afterload on the right ventricle. The boy's clinical condition improved markedly, so he was discharged two weeks after the procedure. The ultimate therapeutic option for medically refractory PAH is LTX or heart-lung transplantation, but because of the short life span after LTX, time was bought by postponing the time of transplantation.

Giant aneurysm in a left coronary artery fistula: diagnostic cardiovascular imaging and treatment considerations.

Congenital coronary artery fistula complicated with giant coronary artery aneurysm is a very rare condition. In this case report, we present a 65-year-old woman, referred to us with a continuous heart murmur, occasional atypical chest pain and few episodes of fainting. A giant aneurysm and a coronary-pulmonary fistula were diagnosed using multiple cardiovascular imaging modalities to provide a sufficient anatomical picture. The patient was considered at high risk of sudden death from aneurysm rupture and received surgical treatment. Subsequent histopathological examination revealed a true aneurysm with severe wall calcifications, ulcerations and large areas with marked thinning of the wall. The postoperative course was uneventful.

Good long-term survival after paediatric heart transplantation.

INTRODUCTION: The brain-death criterion was introduced in Denmark in 1990. The first Danish paediatric heart transplantation (HTx) was performed at Copenhagen University Hospital, Rigshospitalet, in Copenhagen in 1991. We describe our experiences during the first 20 years with paediatric HTx. MATERIAL AND METHODS: This was a retrospective study of 37 paediatric patients (<18 years) who were listed for HTx from 1991 to 2011. RESULTS: A total of 26 of the 37 children listed underwent HTx, nine due to congenital heart disease (CHD) and 17 due to cardiomyopathy (CM). Ten patients died while being on the waiting list. One patient was withdrawn from the list due to spontaneous improvement. A total of 21 patients remain alive. Survival was 92% after five years and 82% after ten years. We had two early (CHD) and three late (CM) deaths. Complications were few, but significant. Early acute rejection occurred in seven patients, whereas one patient with repeated late episodes of acute rejection died from graft failure 5.5 years after HTx. We found a time-related progressive deterioration in renal function. Two patients underwent renal Tx, two others died while being on dialysis. Cardiac allograft vasculopathy occurred in three patients, two of whom died. The third remains alive today, 19 years after HTx. CONCLUSION: Our paediatric HTx results are comparable with those of larger international centres and consistent with true long-term survival. FUNDING: not relevant. TRIAL REGISTRATION: not relevant.

Acute myocardial infarction and syncope in an 18-year-old athlete with an abnormal origin of the left coronary artery: a case report.

We report a case of acute myocardial infarction and syncope in an 18-year-old athlete during high-performance exercise. A coronary arteriography and an angiographic computed tomography scan subsequently revealed a left coronary arterial origin from the right aortic sinus along with an intramural course of the left main stem. The patient was successfully treated with surgical unroofing of the left main stem from inside the aorta. To our knowledge, this is the first report demonstrating this type of anomaly pre- and postoperatively by use of angiographic computed tomography scan in the context of acute coronary syndrome.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) presenting with ventricular fibrillation in an adult: a case report.

Anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA) is a rare congenital anomaly. The usual clinical course is severe left sided heart failure and mitral valve insufficiency presenting during the first months of life. However, in some cases collateral blood supply from the right coronary artery is sufficient and symptoms may be subtle or even absent. Arrhythmias or sudden cardiac death in adult life may be the first clinical presentation in patients with ALCAPA. We report a case, where a 39-year old woman presented with ventricular fibrillation during phycial exertion. Coronary angiography and CT-angiography revealed an anomalous origin of the left coronary artery, and an aortic reimplantation of the left coronary artery was performed followed by ICD implantation. A review of the literature on ALCAPA is presented along with CT images before and after surgery.