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Abstract Background To assess changes in right heart flow and pulmonary artery hemodynamics in patients with repaired Tetralogy of Fallot (rTOF) we used whole heart, four dimensional (4D) velocity mapping (VM) cardiovascular magnetic resonance (CMR). Methods CMR studies were performed in 11 subjects with rTOF (5M/6F; 20.1 ± 12.4 years) and 10 normal volunteers (6M/4F; 34.2 ± 13.4 years) on clinical 1.5T and 3.0T MR scanners. 4D VM-CMR was performed using PC VIPR (Phase Contrast Vastly undersampled Isotropic Projection Reconstruction). Interactive streamline and particle trace visualizations of the superior and inferior vena cava (IVC and SVC, respectively), right atrium (RA), right ventricle (RV), and pulmonary artery (PA) were generated and reviewed by three experienced readers. Main PA net flow, retrograde flow, peak flow, time-to-peak flow, peak acceleration, resistance index and mean wall shear stress were quantified. Differences in flow patterns between the two groups were tested using Fisher's exact test. Differences in quantitative parameters were analyzed with the Kruskal-Wallis rank sum test. Results 4D VM-CMR was successfully performed in all volunteers and subjects with TOF. Right heart flow patterns in rTOF subjects were characterized by (a) greater SVC/IVC flow during diastole than systole, (b) increased vortical flow patterns in the RA and in the RV during diastole, and (c) increased helical or vortical flow features in the PA's. Differences in main PA retrograde flow, resistance index, peak flow, time-to-peak flow, peak acceleration and mean wall shear stress were statistically significant. Conclusions Whole heart 4D VM-CMR with PC VIPR enables detection of both normal and abnormal right heart flow patterns, which may allow for comprehensive studies to evaluate interdependencies of post-surgically altered geometries and hemodynamics.

Objective: We describe experience treating a patient with atrioventricular (AV) discordance with ventriculoarterial (VA) concordance, ventricular septal defect (VSD), and situs inversus. This is a rare congenital lesion in which closing the VSD would septate D-transposition physiology and performing an arterial switch and VSD closure would produce congenitally corrected transposition of the great arteries physiology. As such, it is the only lesion for which an isolated atrial switch (and VSD closure) remains the preferred correction.Case: A term baby girl born with AV discordance/VA concordance, a large VSD, and situs inversus totalis was palliated with atrial septostomy on day of life 3 to allow for optimal mixing and pulmonary artery banding during the 6th week of life to control symptoms of pulmonary overcirculation and protect the pulmonary vasculature. At 8 months of age, she underwent complete repair with a Senning atrial switch procedure, VSD closure, and pulmonary artery debanding with pulmonary arterioplasty.Results: The patient underwent corrective surgery with patch closure of the VSD, and the Senning atrial switch procedure resulting in a total anatomic correction. The patient was discharged on postoperative day 6 and is clinically doing well 12 months later. Follow-up transthoracic echocardiogram shows no pulmonary venous baffle obstruction, mild systemic venous baffle obstruction, and moderate pulmonary stenosis/pulmonary insufficiency.Conclusion: The isolated atrial switch is rarely employed as its outcomes are inferior to the arterial switch operation in the setting of the dextro-transposition of the great arteries. However, it remains the procedure of choice for AV discordance with VA concordance as it leads to physiologically corrected biventricular circulation. [ABSTRACT FROM AUTHOR]

There is growing appreciation for inherited structural heart diseases and their genetic causes. One causal gene for congenital cardiac and vascular lesions is FLNA which encodes a critical protein for cytoskeletal and extracellular matrix development. A newborn infant male, with prenatally diagnosed polyvalvular dysfunction, presented with low cardiac output and postnatally detected aortic arch hypoplasia and coarctation. Attempted palliative coarctation intervention resulted in vascular complications that ultimately contributed to his demise. This case report highlights polyvalvular dysplasia, vascular abnormalities, and a likely causal de novo missense variant in the FLNA gene (c.5180 C>T p.P1727L) not previously described., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2024 The Authors.)

Background: The etiology of isolated right ventricular hypertrophy (RVH) is distinct from other forms of hypertrophic cardiomyopathy. RVH is typically seen in the setting of pulmonary valve stenosis or Tetralogy of Fallot. A rare cause of isolated RVH is premature closure of the patent ductus arteriosus (PDA) in utero that results in pulmonary hypertension. This can have a range of outcomes, from spontaneous resolution to fetal demise., Methods: This case report describes a term infant who presented with respiratory distress and striking isolated RVH, pulmonary hypertension, and no PDA. She was treated conservatively with supplemental oxygen., Results: The patient was gradually weaned off oxygen over the course of two weeks and follow-up echocardiography showed resolution of her RVH and pulmonary hypertension by 14 weeks of age., Conclusions: The presentation and course of this patient with severe isolated RVH is consistent with spontaneous premature closure of the ductus arteriosus in utero.