Percutaneous closure of a sinus venosus defect with partial anomalous pulmonary venous return.

A 73-year-old man with a history of hypertension, hyperlipidemia, and obesity presented for cardiovascular evaluation. He was experiencing mild fatigue and dyspnea on exertion. Transthoracic echocardiogram (TTE) showed right ventricular dilation, which was otherwise unremarkable.

Early Outcomes From a Multicenter Transcatheter Self-Expanding Pulmonary Valve Replacement Registry.

BACKGROUND: Transcatheter pulmonary valve replacement (TPVR) with the self-expanding Harmony valve (Medtronic) is an emerging treatment for patients with native or surgically repaired right ventricular outflow tract (RVOT) pulmonary regurgitation (PR). Limited data are available since U.S. Food and Drug Administration approval in 2021. OBJECTIVES: In this study, the authors sought to evaluate the safety and short-term effectiveness of self-expanding TPVR in a real-world experience. METHODS: This was a multicenter registry study of consecutive patients with native RVOT PR who underwent TPVR through April 30, 2022, at 11 U.S. CENTERS: The primary outcome was a composite of hemodynamic dysfunction (PR greater than mild and RVOT mean gradient >30 mm Hg) and RVOT reintervention. RESULTS: A total of 243 patients underwent TPVR at a median age of 31 years (Q1-Q3: 19-45 years). Cardiac diagnoses were tetralogy of Fallot (71%), valvular pulmonary stenosis (21%), and other (8%). Acute technical success was achieved in all but 1 case. Procedural serious adverse events occurred in 4% of cases, with no device embolization or death. Hospital length of stay was 1 day in 86% of patients. Ventricular arrhythmia prompting treatment occurred in 19% of cases. At a median follow-up of 13 months (Q1-Q3: 8-19 months), 98% of patients had acceptable hemodynamic function. Estimated freedom from the composite clinical outcome was 99% at 1 year and 96% at 2 years. Freedom from TPVR-related endocarditis was 98% at 1 year. Five patients died from COVID-19 (n = 1), unknown causes (n = 2), and bloodstream infection (n = 2). CONCLUSIONS: In this large multicenter real-world experience, short-term clinical and hemodynamic outcomes of self-expanding TPVR therapy were excellent. Ongoing follow-up of this cohort will provide important insights into long-term outcomes.

Fetal Arrhythmia Leading to a Diagnosis of Congenital Long QT Syndrome Type II.

We describe the case of an asymptomatic young pregnant woman with a diagnosis of congenital long QT syndrome type II in the context of in utero fetal 2:1 heart block and ventricular tachycardia. The presentation, clinical considerations, and management of the mother and baby in the antepartum and postpartum periods are discussed.

Challenges in Accurate Diagnosis of HCC in FALD: A Case Series.

Patients with single ventricle cardiac disease palliated with Fontan procedures inevitably develop Fontan-Associated Liver Disease (FALD), which poses a significant risk for hepatocellular carcinoma (HCC). Standard imaging criteria for the diagnosis of cirrhosis are not reliable due to parenchymal heterogeneity of FALD. We present 6 cases to demonstrate our center's experience and the challenges in diagnosing HCC within this patient population.

Massive Right Ventricle Enlargement in Unrepaired Sinus Venosus Atrial Septal Defect: Repair or Not?

Sinus venosus atrial septal defect (SVASD) with partial anomalous pulmonary venous return is a rare congenital heart defect that may present in adulthood when right heart enlargement has already occurred. We describe a case of unrepaired sinus venosus atrial septal defect and partial anomalous pulmonary venous return with massive right heart enlargement and recurrent atrial arrhythmias.

Correlation of transthoracic echocardiography-derived pulmonary to systemic flow ratio with hemodynamically estimated left to right shunt in atrial septal defects.

Background: Transthoracic echocardiographic (TTE) estimation of the pulmonary to systemic flow ratio (Qp/Qs) is routinely used in clinical practice and is included in the American Society of Echocardiography Guidelines. We sought to assess its real-world applicability with a particular focus on hemodynamically significant shunt lesions. Methods: Retrospective single institutional review of TTE's in patients with secundum atrial septal defect prior to cardiac catheterization (cath) from 2012 to 2018 was performed (n = 109), those with technically limited images for Qp/Qs calculation (n = 11) and those with time interval between TTE and cath >60 days were excluded (n = 14). Qp/Qs was calculated from stored clips by previously described methods and correlated with those obtained by oximetry. Patients were subdivided into two age groups <21 (Group 1) and ≥22 years (Group 2). TTE and cath methods for Qp/Qs estimation were compared using paired t-test, Pearson's correlation coefficient, and Bland-Altman plots. Results: Eighty-four subjects met inclusion criteria (age range 3-78 years). Group 1 n = 35; median age 10 years; Group 2 n = 49; median age 49 years. Transthoracic echocardiogram was performed 19.5 ± 15 days prior to cath. Mean Qp/Qs derived by cath and TTE were 2.09 ± 0.9 versus 2.54 ± 1.2 (P < 0.0001). Overall correlation was poor between the methods (r 2 = 0.32, P < 0.0001) and continued to be poor for Groups 1 and 2 (r 2= 0.24, P = 0.003 and r 2= 0.40, P < 0.0001 respectively). Bland-Altman plots demonstrated poor agreement between the predetermined limits of agreement (-0.5-1.5). Conclusion: Transthoracic echocardiography estimated Qp/Qs, although routinely utilized in clinical practice, has poor correlation and agreement with oximetry-derived Qp/Qs. The test performs poorly in all age groups in detecting a hemodynamically significant shunt and tends to overestimate the degree of left to right shunt.

Ventricular septal defect Doppler peak gradient underestimates right ventricular systolic pressure in the presence of right bundle branch block.

Estimation of the right ventricular systolic pressure (RVSP) using echocardiographically derived maximal instantaneous gradient across the ventricular septal defect is a well-utilized tool, which can potentially underestimate the RVSP in patients with conduction abnormalities as such its utility in these patients is questionable.

To Be or Not to Be Eisenmenger: The Different Shades of Blue.

Eisenmenger syndrome refers to any untreated congenital cardiac defect with an intracardiac communication that leads to pulmonary arterial hypertension, reversal of intracardiac shunting, and cyanosis. We describe a 40-year-old cyanotic patient with congenital heart disease with presumed Eisenmenger syndrome who was considered inoperable. Testing revealed a partial atrioventricular septal defect with no evidence of pulmonary arterial hypertension, and the patient underwent successful cardiac repair. (Level of Difficulty: Intermediate.).

COVID-19 in an Adult With Tricuspid Atresia S/P Fontan Palliation.

In December 2019, a pathogenic novel human coronavirus (HCoV), termed SARS-CoV-2, was recognized in Wuhan, China, causing significant morbidity and mortality. The illness caused by SARS-CoV-2 is labelled coronavirus disease-2019 (COVID-19) by the World Health Organization. We report the first case of COVID-19 in an adult congenital heart disease patient with single ventricle physiology S/P Fontan palliation. (Level of Difficulty: Advanced.).

ASSURED clinical study: New GORE® CARDIOFORM ASD occluder for transcatheter closure of atrial septal defect.

OBJECTIVE: Assess clinical performance of a new device for transcatheter closure of atrial septal defect (ASD). BACKGROUND: Previously-approved ASD Closure devices have known limitations. Device erosion has been associated with the AMPLATZER® septal occluder in patients with retro-aortic rim deficiency (<5 mm), while defects ≥18 mm are too large for the GORE® CARDIOFORM septal occluder. The GORE® CARDIOFORM ASD occluder (GCA), a hybrid of the approved devices, was designed to expand the eligible ASD population. METHODS: One-hundred and twenty-five ASD patients were enrolled in a prospective, multicenter registry. Descriptive clinical endpoints included: technical implant success, closure success, serious adverse events (SAE), clinically significant new arrhythmia, and wire frame fracture. Procedural outcomes and adverse events were adjudicated by an Echocardiography Core Lab and Independent Data Review Board, respectively. RESULTS: Median subject age was 12.3 years (range 2.9-84.7), with 72% of patients ≤18 years old. Median ASD stop-flow diameter was 17.0 mm (8.0-30.0), with 43% ≥18 mm. Deficient retro-aortic rim occurred in 57% of subjects, and 30% had both diameter ≥ 18 mm and deficient rim. Technical Implant Success was achieved in 120/125 (96%), though three devices were removed within 24 hr. At the scheduled 6-month evaluation, 112/117 returned for evaluation. All 112 had closure success. SAE occurred in 6/125 (4.8%) and 6/125 (4.8%) had clinically significant new arrhythmia. Wire frame fractures occurred in 37/104 (36%), without any associated clinical sequelae, residual shunt, or device instability. CONCLUSION: The GCA performed effectively and safely in this initial cohort, and led to FDA approval based on this data.

Congenitally "Inverted" Pulmonary Valve in Tetralogy of Fallot: When Nature Falters.

A cyanotic neonate with tetralogy of Fallot was found to have a congenitally inverted pulmonary valve. Diagnosis was made via echocardiography and cardiac catheterization. The valve opened retrograde into the right ventricle, which allowed severe regurgitation and prevented anterograde flow. This report is the first description of this anomaly in medical literature. (Level of Difficulty: Intermediate.).

Frequency of Ventricular Arrhythmias and Other Rhythm Abnormalities in Children and Young Adults With the Marfan Syndrome.

Patients with the Marfan syndrome (MFS) are at risk for sudden death. The contribution of arrhythmias is unclear. This study examines the prevalence of arrhythmias in children with the MFS and their relation to clinical and/or echocardiographic factors. Data from the Pediatric Heart Network randomized trial of atenolol versus losartan in MFS were analyzed (6 months to 25 years old, aortic root diameter z-score > 3.0, no previous aortic surgery and/or dissection). Baseline 24-hour ambulatory electrocardiographic monitoring was performed. Significant ventricular ectopy (VE) and supraventricular ectopy (SVE) were defined as ≥10 VE or SVE/hour, or the presence of high-grade ectopy. Three-year composite clinical outcome of death, aortic dissection, or aortic root replacement was analyzed. There were 274 analyzable monitors on unique patients from 11 centers. Twenty subjects (7%) had significant VE, 13 (5%) significant SVE; of these, 2 (1%) had both. None had sustained ventricular or supraventricular tachycardia. VE was independently associated with increasing number of major Ghent criteria (odds ratio [OR] = 2.13/each additional criterion, p = 0.03) and greater left ventricular end-diastolic dimension z-score (OR = 1.47/each 1 unit increase in z-score, p = 0.01). SVE was independently associated with greater aortic sinotubular junction diameter z-score (OR = 1.56/each 1 unit increase in z-score, p = 0.03). The composite clinical outcome (14 events) was not related to VE or SVE (p ≥ 0.3), but was independently related to heart rate variability (higher triangular index). In conclusion, in this cohort, VE and SVE were rare. VE was related to larger BSA-adjusted left ventricular size. Routine ambulatory electrocardiographic monitoring may be useful for risk stratification in select MFS patients.

A multicenter review of ablation in the aortic cusps in young people.

BACKGROUND: Ablation within the aortic cusp is safe and effective in adults. There are little data on aortic cusp ablation in the pediatric literature. We investigated the safety and efficacy of aortic cusp ablation in young patients. METHODS: A retrospective, descriptive study of aortic cusp ablation in five pediatric electrophysiology centers from 2008 to 2014 was performed. All patients <21 years of age who underwent ablation in the aortic cusps were included. Factors analyzed included patient demographics, procedural details, outcomes, and complications. RESULTS: Thirteen patients met inclusion criteria (median age 16 years [range 10-20.5] and median body surface area 1.58 m2 [range 1.12-2.33]). Substrates for ablation included: nine premature ventricular contractions or sustained ventricular tachycardia (69%), two concealed anteroseptal accessory pathways (APs) (15%), one Wolff-Parkinson-White with an anteroseptal AP (8%), and one ectopic atrial tachycardia (8%). Three-dimensional electroanatomic mapping in combination with fluoroscopy was used in 12/13 (92%) patients. Standard 4-mm-tip radiofrequency (RF) current was used in 11/13 (85%) and low-power irrigated-tip RF in 2/13 (15%). Angiography was used in 13/13 and intracardiac echocardiography was additionally utilized in 3/13 (23%). Ablation locations included: eight noncoronary (62%), three left (23%), and two right (15%) cusps. Ablation was acutely successful in all patients. At median follow-up of 20 months, there was one recurrence of PVCs (8%). There were no ablation-related complications and no valvular injuries observed. CONCLUSION: Arrhythmias originating from the coronary cusps in this series were successfully and safely ablated in young people without injury to the coronary arteries or the aortic valve.