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Variant coronary anatomy (VarCA) is frequent in D-Transposition of the great arteries (d-TGA). There are a paucity of data on the effect of the VarCA on the exercise capacity (XC) in patients with repaired d-TGA. This retrospective study included patients with d-TGA who underwent an arterial switch operation (ASO) and had at least one cardiopulmonary exercise test (CPET). Data from the treadmill CPET and simultaneously performed spirometry were collected. The parameters of CPET were compared between patients with usual anatomy vs. VarCA. Longitudinal changes in XC in patients with ASO were also analyzed. A total of 44 patients with either usual coronary anatomy (n = 27, 61%) or VarCA (n = 17, 39%) met inclusion criteria. There was no significant difference in oxygen consumption (%VO

Vascular ring is a rare congenital anomaly in which the abnormal origin of the aorta or its branches and pulmonary arteries leads to encircling and compression of the trachea and esophagus. A right aortic arch (RAA) with an aberrant left subclavian artery is one of the most common forms of vascular ring. Here, we report a case of a prenatally diagnosed vascular ring resulting from an RAA with an aberrant left subclavian artery. When the infant was 7 months of age, the development of noisy breathing prompted further evaluation with cardiac magnetic resonance imaging that showed an atretic left subclavian artery associated with collateral retrograde flow from the left vertebral artery to the distal portion of the subclavian artery. Our findings indicate that an untreated RAA with an aberrant left subclavian artery may be associated with an increased risk of developing subclavian artery steal syndrome.

Patient prosthesis mismatch (PPM) is an important factor of the outcome in transcatheter aortic valve implantation. However, the impact of PPM in transcatheter pulmonic valve implantation (TPVI) has not been studied. Based on the narrowest valve stent diameters in two views of fluoroscopy, internal geometric orifice area (GOA) of the valve stent was calculated and indexed by body surface area (BSA), deriving iGOA. To define PPM in TPVI, receiver operating characteristics (ROC) curve analysis for iGOA for predicting significant residual right ventricular outflow tract (RVOT) gradient was used to derive the optimal cut-off value of iGOA. Our cohort were divided into 2 groups: PPM versus non-PPM. The clinical data were compared between 2 groups. TPVI was performed using Melody valve in 101 patients. Significant RVOT residual pressure gradient (≥ 15 mmHg) was observed in 31 patients (39.6%). Over a mean follow up periods of 6.9 ± 2.7 years, 22 patients (21.8%) required repeat interventions (16 transcatheter, 11 surgical, and both in 5 patients). Based on the ROC analysis, the best cut-off value of iGOA was 1.25 cm2/m2 (area under the curve 0.873, p

Reduced exercise capacity and restrictive lung physiology are common in patients after Fontan palliation (FP). However, there is paucity of data regarding the association between specific spirometry patterns and key exercise parameters in this population. This is a single-center, cross-sectional, study correlating pulmonary function and exercise parameters in children with FP. Patients who were ≤ 18 years of age and underwent a comprehensive cardiopulmonary treadmill exercise stress test (CPT) and spirometry at the same time, were included. Patients were categorized as (i) normal or (ii) abnormal based on the results of spirometry. The abnormal group was subdivided into (a) restrictive, (b) obstructive, and (c) mixed patterns. Demographic and key exercise parameters were compared between groups. Our study included 82 patients who underwent CPT at 13.6 (IQR, 11.3–15.4) years of age. A reduced exercise capacity (%VO2 ≤ 85%) was noted in the majority (n = 50, 61%). Spirometry was abnormal in 47 (57%) patients [restrictive (n = 25, 30%), obstructive (n = 12, 15%), and mixed (n = 10, 12%)]. The abnormal spirometry group had significant lower %VO2 (77% vs. 92%, p = 0.01) and METS (8.4 vs. 9.6, p = 0.02). Subgroup analysis revealed that obstructive (p = 0.04) and mixed (p = 0.02) patterns were associated with a significant decrease in % VO2. Majority of the children demonstrated an abnormal spirometry pattern post-FP. Abnormal pulmonary function was associated with the reduced exercise capacity. Identification and treatment of the abnormal lung function may improve the exercise capacity in these patients and improve the morbidity.

We report a neonate with dilated cardiomyopathy and have echocardiographic findings consistent with “functional” tricuspid atresia. There was an echo-bright, plate-like tissue at the tricuspid valve position with no forward flow across it. This report underscores the role of right ventricle intracavitary haemodynamic influence on the tricuspid valve leaflet excursion and demonstrates a phenomenon of “pseudo or functional tricuspid atresia” mimicking tricuspid atresia in a patient with acute presentation of cardiomyopathy.

Left ventricular outflow tract obstruction in patients with pulmonary atresia with intact ventricular septum has been rarely reported. Data are lacking on the impact and management of systemic ventricular outflow tract obstruction that developed following the Fontan procedure. We report a case of an 8-year-old male who developed left ventricular outflow tract obstruction 6 months after the Fontan procedure.

Conduit stenosis is a major, albeit rare, complication following the Fontan palliation. A single-baffle conduit with polytetrafluoroethylene is widely used for an extracardiac type Fontan palliation. A polyethylene terephthalate conduit (Dacron) is sometimes used for the conduit when more flexibility is required. A Y-shaped conduit is rarely used, but it may reduce the energy loss and achieve better hepatic flow distribution. Data on the long-term patency and complications when using a Y-shaped Dacron conduit is lacking. We report a case of a severely stenotic Y-shaped Dacron conduit in a patient who underwent extracardiac Fontan palliation.

A 3-month-old female was diagnosed at 1 month of age with DeSanto-Shinawi syndrome (DSS) and bicuspid aortic valve with trivial stenosis. The aortic valve stenosis progressed to severe within 2 months and required balloon aortic valvuloplasty. This is the first case of aortic stenosis (AS) associated with DSS, and the syndrome may be the reason for the rapid worsening of AS in this case.

Rhabdomyomas are the most common paediatric cardiac tumours. The natural history of these tumours is mostly benign, and the tumour usually regresses spontaneously. Although surgical resection of these tumours is one of the considerations in patients with ventricular outflow obstruction, a palliation with Blalock–Taussig shunt is an alternative approach with the hope of regression of the tumour over time. We report a case of prenatally diagnosed rhabdomyomas in the right ventricle and its outflow presenting as hemodynamic simulating hypoplastic right ventricle in a newborn. She required prostaglandin and Blalock–Taussig shunts palliation for pulmonary flow and subsequent regression of tumours.

The echogenic mass in the pulmonary artery is a rare finding in newborns. Differential diagnoses include pulmonary artery thrombosis, ductal aneurysm, and malignant tumor. We report a newborn case who presented with mild desaturation and was found to have a large echogenic mass at the pulmonary bifurcation on echocardiography, which caused partial flow obstruction in the proximal left pulmonary artery. Along with the findings of cardiac computed tomography and magnetic resonance imaging, the diagnosis of ductus arteriosus aneurysm (DAA) was made. This DAA spontaneously regressed without any intervention at 4 months follow-up.

Background: Blood pressure (BP) response to the treadmill cardiopulmonary exercise test (CPET) has yet been studied with no standard criteria for abnormal BP responses in children. Our hypothesis was that the reference values of BP responses to the treadmill CPET should be defined by gender and age in children. Objectives: The objective of our study was to describe the BP responses to the treadmill CPET and propose the criteria for abnormal BP responses in children. Methods: The treadmill CPET data were retrospectively analyzed to evaluate the BP responses to exercise during the period of 8 years (2011-2018). Children aged Results: Our cohort consisted of 1,085 children (males 59%) aged 7-17 years. The analysis of variance for Systolic BP at peak exercise (peak SBP) and change (Δ) of SBP showed significant age and gender effects and age-gender interaction effect ( p p 90% confidence interval upper limit of reference values based on age and gender. Blunted BP response was defined by 1) ΔSBP Conclusion: Blood Pressure response to the treadmill CPET was dependent on age and gender in children with normal hearts. The proposed criteria for abnormal BP responses in children should prove useful in interpreting BP responses in children during the CPET.

The surgical options for significant aortic valve disease include either Ross procedure (RP) or aortic valve replacement (AVR). The exercise stress test is routinely performed in these patients to assess the objective functional capacity. This retrospective study was conducted to evaluate the differences and the longitudinal changes of exercise capacity in patients following the RP and AVR for aortic valve disease. This is an IRB approved retrospective study and included patients who had either RP or AVR performed for aortic valve disease and had at least one exercise stress test performed after the surgical procedure. Patients with other congenital heart disease, pacemaker or defibrillators, and those with inadequate data were excluded. Demographic data including age at surgery, type of surgery and type of aortic valve was collected. Data regarding treadmill cardiopulmonary exercise test (CPET) was also collected. A total of 47 patients met inclusion criteria and were equally represented in each group, i.e. RP [n = 23, 73.9% male, age at surgery 11.2 (4.5–15.9) years] vs. AVR [n = 24, 88% mechanical AVR, 60.9% male, age at surgery 15.1 (12.8–19.4) years]. There was a significant decline in predicted oxygen consumption (%VO2) at time of first post-operative CPET in patients after AVR compared to RP (79 vs. 88%, p = 0.048) over a similar accrued median interval follow-up (4.6 vs. 6.2 years, p = 0.2). The longitudinal follow-up analysis of following AVR (n = 11, 54.5% male, median inter-test duration of 5 years) showed significant decline in peak exercise capacity or VO2 (34.2 vs. 26.2 vs., p = 0.006). In contrast, after RP (n = 12 patients [58.3% male, median inter-test duration 7.1 of years], exercise capacity and other key parameters remained preserved. In this small sentinel study, we report a better initial exercise capacity among patients after RP compared to AVR over an intermediate follow-up. During longitudinal follow-up in a subset of patients, exercise capacity remained preserved amongst the RP group while it further declined in the AVR group.