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.

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.

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.

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.

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.

How do we define congenital heart defects for scientific studies?

Estimates of the prevalence of congenital heart defects (CHD) have been published over many years and from many regions. As they are based on different definitions of which cases to include in the CHD prevalence, published prevalence estimates vary substantially. With the increasing use of echocardiography in neonatal intensive care, a patent ductus arteriosus (PDA) or flow over the atrial septum will often be visible. These findings may be coded as CHD at discharge and in this way falsely increase the CHD prevalence in the population. There are several purposes for which population-based data on CHD may be used: etiology, planning of treatment, or obtain information on outcome, including mortality. For etiology studies, it is important to include terminations of pregnancy as well as all births with CHD. For mortality studies in live births, inclusion of preterm born infants with PDA will increase overall mortality of CHD. The Danish Register of Congenital Heart Disease is based on hospital discharge diagnoses and diagnoses from outpatient visits. To increase the validity of these data, extensive data cleaning has been carried out based on record review and knowledge on the discharge coding practice. We include PDA and atrial septal defects as CHD cases if these defects are still open 2 months after birth. International consensus on how to define CHD would improve the validity and comparability of epidemiological studies on CHD.

Cardiac surgery in grown-up congenital heart patients. Will the surgical workload increase?

The number of patients with grown-up congenital heart (GUCH) disease is steadily increasing. Although there is agreement that the medical service for GUCH patients should be expanded in coming years, it is still unknown whether this should also include the surgical service. In an attempt to elucidate this we reviewed our population of surgical GUCH patients (n=225) operated in our institution from 1998 to 2005. The patients' charts were reviewed. For details of the procedures, the hospital's internal database (PATS) was used. Patients were stratified according to diagnosis and complexity of the surgical procedures in a simple, moderate and complex category group. The yearly number of operations remained stable in the period. However, whereas the size of the simple complexity group was reduced in the study period, the size of both the moderate and the complex category groups increased progressively. Forty-four percent of the surgical procedures were reoperations. Morbidity and mortality (1.3%) were low. The present study suggests that the future surgical GUCH patients will be increasingly complex. However, it is speculative whether the total number of surgical GUCH patients will increase. This is especially explained by the continuous introduction of new percutaneous catheter techniques.

[Aortic coarctation in a teenager presenting as mycotic pseudoaneurysm]. / Coarctatio aortae debuterende som mykotisk pseudoaneurisme hos en teenager.

Since the introduction of surgical treatment for coarctation aortae, presentation of the disease with postcoarctation mycotic pseudoaneurysm has become rare. We report an 18-year-old male with endarteritis caused by Staphylococcus aureus treated with appropriate antibiotics. Later the patient developed an irritative cough and problems with swallowing. CT and MRI of the chest demonstrated a postcoarctation pseudoaneurysm. After acute surgery with resection of the coarctation and pseudoaneurysm and insertion of a tube graft, the patient had an uneventful postsurgical course.

Pressure-pain threshold in human temporal region. Evaluation of a new pressure algometer.

A hand-held pressure algometer with a pressure sensitive strain gauge at the tip was used to measure the pressure-pain threshold (PPT) in the temporal region of healthy volunteers. Various sizes of circular tips and various application rates were tested before selecting an area of 0.5 cm2 and a constant application rate of 0.68 N X sec-1 for future use. A highly significant correlation was found between PPT values obtained from the two sides (of the head) (P less than 0.001) and between PPT values obtained with a 3-week interval (P less than 0.001). In a series of 50 immediate consecutive measurements in the same individual, the mean PPT was 171 kPa (N = 6, 2 S.D. 24%). The mean relative change in PPT after a 3-week interval was 0 +/- 51% (N = 11, 2 S.D.). In the course of 5 repeated determinations at weekly intervals there was a significant increase in PPT (ANOVA, P less than 0.05). Subcutaneous lignocaine significantly elevated PPT compared to placebo. Due to the high inter-individual variation, determinations of PPT for group comparisons should include rather large population samples, whereas in paired studies, the intra-individual variation allows the investigation of much smaller groups (10-20 subjects). It is our experience that the pressure algometer is easy to operate in the hands of a skilled laboratory assistant.