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Byline: M. Idrees, K. Al-Najashi, A. Khan, S. Al-Dammas, H. Al-Awwad, E. Batubara, A. Al Otai, J. Abdulhameed, A. Fayed, T. Kashour, SAPH Registry Taskforce. Aims: The main objective of [...]

Background: Altered coagulation is a striking feature of COVID-19. Adult patients with congenital heart disease (ACHD) are prone to thromboembolic (TE) and bleeding complications., Objectives: The purpose of this study was to investigate the prevalence and risk factors for COVID-19 TE/bleeding complications in ACHD patients., Methods: COVID-19-positive ACHD patients were included between May 2020 and November 2021. TE events included ischemic cerebrovascular accident, systemic and pulmonary embolism, deep venous thrombosis, myocardial infarction, and intracardiac thrombosis. Major bleeding included cases with hemoglobin drop >2 g/dl, involvement of critical sites, or fatal bleeding. Severe infection was defined as need for intensive care unit, endotracheal intubation, renal replacement therapy, extracorporeal membrane oxygenation, or death. Patients with TE/bleeding were compared to those without events. Factors associated with TE/bleeding were determined using logistic regression., Results: Of 1,988 patients (age 32 [IQR: 25-42] years, 47% male, 59 ACHD centers), 30 (1.5%) had significant TE/bleeding: 12 TE events, 12 major bleeds, and 6 with both TE and bleeding. Patients with TE/bleeding had higher in-hospital mortality compared to the remainder cohort (33% vs 1.7%; P  < 0.0001) and were in more advanced physiological stage ( P  = 0.032) and NYHA functional class ( P  = 0.01), had lower baseline oxygen saturation ( P  = 0.0001), and more frequently had a history of atrial arrhythmia ( P  < 0.0001), previous hospitalization for heart failure ( P  < 0.0007), and were more likely hospitalized for COVID-19 ( P  < 0.0001). By multivariable logistic regression, prior anticoagulation (OR: 4.92; 95% CI: 2-11.76; P  = 0.0003), cardiac injury (OR: 5.34; 95% CI: 1.98-14.76; P  = 0.0009), and severe COVID-19 (OR: 17.39; 95% CI: 6.67-45.32; P  < 0.0001) were independently associated with increased risk of TE/bleeding complications., Conclusions: ACHD patients with TE/bleeding during COVID-19 infection have a higher in-hospital mortality from the illness. Risk of coagulation disorders is related to severe COVID-19, cardiac injury during infection, and use of anticoagulants., Competing Interests: The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (© 2023 The Authors.)

Fontan palliation represents one of the most remarkable surgical advances in the management of individuals born with functionally univentricular physiology. The operation secures adult survival for all but a few with unfavourable anatomy and/or physiology. Inherent to the physiology is passive transpulmonary blood flow, which produces a vulnerability to adequate filling of the systemic ventricle at rest and during exertion. Similarly, the upstream effects of passive flow in the lungs are venous congestion and venous hypertension, especially marked during physical activity. The pulmonary vascular bed has emerged as a defining character on the stage of Fontan circulatory behaviour and clinical outcomes. Its pharmacologic regulation and anatomic rehabilitation therefore seem important strategic therapeutic targets. This review seeks to delineate the important aspects of pulmonary artery development and maturation in functionally univentricular physiology patients, pulmonary artery biology, pulmonary vascular reserve with exercise, and pulmonary artery morphologic and pharmacologic rehabilitation.

Background: Adults with congenital heart disease (CHD) have been considered potentially high risk for novel coronavirus disease-19 (COVID-19) mortality or other complications., Objectives: This study sought to define the impact of COVID-19 in adults with CHD and to identify risk factors associated with adverse outcomes., Methods: Adults (age 18 years or older) with CHD and with confirmed or clinically suspected COVID-19 were included from CHD centers worldwide. Data collection included anatomic diagnosis and subsequent interventions, comorbidities, medications, echocardiographic findings, presenting symptoms, course of illness, and outcomes. Predictors of death or severe infection were determined., Results: From 58 adult CHD centers, the study included 1,044 infected patients (age: 35.1 ± 13.0 years; range 18 to 86 years; 51% women), 87% of whom had laboratory-confirmed coronavirus infection. The cohort included 118 (11%) patients with single ventricle and/or Fontan physiology, 87 (8%) patients with cyanosis, and 73 (7%) patients with pulmonary hypertension. There were 24 COVID-related deaths (case/fatality: 2.3%; 95% confidence interval: 1.4% to 3.2%). Factors associated with death included male sex, diabetes, cyanosis, pulmonary hypertension, renal insufficiency, and previous hospital admission for heart failure. Worse physiological stage was associated with mortality (p = 0.001), whereas anatomic complexity or defect group were not., Conclusions: COVID-19 mortality in adults with CHD is commensurate with the general population. The most vulnerable patients are those with worse physiological stage, such as cyanosis and pulmonary hypertension, whereas anatomic complexity does not appear to predict infection severity., Competing Interests: Funding Support and Author Disclosures The authors have reported that they have no relationships relevant to the contents of this paper to disclose., (Copyright © 2021 American College of Cardiology Foundation. Published by Elsevier Inc. All rights reserved.)

Background: Fontan outcomes data from large volume Middle Eastern Centres are lacking. We report our experience after the Fontan operation from a tertiary cardiac centre in Saudi Arabia., Method: All 458 consecutive patients who had Fontan surgery 1986 through 2015 at the Prince Sultan Cardiac Centre, Riyadh [PSCC], Saudi Arabia, were evaluated for baseline, early and late post-operative outcomes and their uni and multivariate determinants., Results: The mean age at Fontan operation was 7 years [IQR 4.8-9.0]. The most common anatomic diagnoses were tricuspid atresia (104 [23%]) and double-inlet left ventricle (81 [18%]). Only 3 patients in the present series had hypoplastic left heart syndrome [HLHS]. Early mortality [i.e. during Fontan surgical admission] was 3.1%. At late follow-, 35 (8%) patients were lost to follow up. The 1, 5, 10, 20 and 30 year survival was 96%, 94%, 93% and 85%, respectively. In the modern surgical era, 5, 10 and 15 year survival were 96%, 95% and 93% respectively. Univariate determinants of death or transplant were hypoalbuminemia, elevated NtProBNP >500, surgical era prior to 1999, the lack of Fontan fenestration, and prior atriopulmonary Fontan [APF] procedure. On multivariate analysis, surgical era before 1999 and prior APF procedure were independently associated with death or transplant., Conclusions: Fontan patients from this large volume Middle Eastern centre have comparable early and late mortality outcomes compared to prior published reports. Rigorous selection criteria at the time of Fontan, and Fontan specific dedicated care teams are likely contributors to this success., Competing Interests: Declaration of Competing Interest The research has no conflict of interest and is not funded through any source., (Copyright © 2020 Elsevier B.V. All rights reserved.)

Background: Fetal aortic stenosis may progress to hypoplastic left heart syndrome (HLHS), which carries a poor prognosis. We report two infants with fetal aortic stenosis successfully treated with fetal aortic valvuloplasty (FAV) using balloon dilatation., Case Presentation: Of five fetuses with aortic stenosis fulfilling the FAV criteria of severe aortic stenosis with a left ventricular length Z-score of ≥ - 2, retrograde flow in the transverse aortic arch, left-to-right flow across the foramen ovale, monophasic mitral inflow, and significant left ventricular dysfunction, we obtained permission for FAV in two fetuses. FAV was performed successfully under echocardiographic guidance using balloon dilatation. Both fetuses survived to birth. During FAV, mild pericardial effusion developed when introducing the stylet needle in the second fetus, and this resolved within 48 h. No intraprocedural complications occurred in the first patient, and no maternal complications occurred. The first infant underwent the Ross procedure after birth and is currently 7 years old and doing well. The second patient underwent aortic and mitral valve repair with endocardial fibroelastosis resection approximately 2 weeks after birth, which temporarily addressed the mitral valve stenosis; high doses of inotropes were subsequently required. The infant died of sepsis at 2 months of age., Conclusion: FAV using balloon dilatation to treat fetal aortic stenosis was successful in our two patients, with subsequent neonatal biventricular repair resulting in long-term survival in one patient and death secondary to sepsis in the second patient.

Background: Aortic pseudoaneurysms are rare but life-threatening complications usually seen after cardiac surgery. The causes could be multifactorial such as infection or trauma., Case Presentation: We report the surgical management of a postoperative pseudoaneurysm of the ascending aorta caused by methicillin-resistant Staphylococcus aureus in a 2-year-old Middle Eastern boy who had undergone ventricular septal defect closure, subaortic membrane resection, and pulmonary artery de-banding. He was immediately operated on for resection of the aneurysm. A computed tomography scan at 2 months following surgery showed no aneurysm. Antibiotics were continued for 6 weeks and our patient was discharged with negative blood cultures., Conclusion: Early diagnosis and appropriate treatment of such rare complication can be lifesaving.

Objectives: This article sought to describe a new modification of the Melody valve that allows percutaneous pulmonary valve implantation (PPVI) in complex outflow tracts., Background: PPVI has been validated as a valuable therapeutic option for the management of patients with dysfunctional right ventricular outflow tracts (RVOT). However, complex and unfavourable RVOT anatomy continue to limit the indications for PPVI., Methods: Between April 2012 and November 2013, PPVI was performed in 10 patients (mean age = 16, 3 ± 5 years old) using a new modification of the Melody® valve consisting in a manual shortening of the Melody by folding the two extremities of the stent. We reviewed the results of this technique., Results: Indications were short RVOT in three patients, prevention of retrosternal compression in two patients, bioprosthetic valves in four and coronary arteries proximity in one. No complication occurred during procedures. All patients had excellent hemodynamic results [mean post PPVI RV-PA gradient was 14 ± 6 mm Hg, three patients had trivial pulmonary regurgitation (PR) and the remaining had no PR]. After a mean follow-up of 11 months (range 5-21 months), no patient had reintervention. No valve dysfunction or stent fractures were observed., Conclusion: The "Folded valve technique" is a safe modification of the Melody valve. By shortening the valve, this technique allowed PPVI in short and complex RVOTs with vulnerable neighborhood., (© 2014 Wiley Periodicals, Inc.)

We describe a case with pacemaker implantation for cardiac resynchronization therapy (CRT) in a patient with complex congenital heart disease, facilitated by cardiac computed tomography (CT) and coronary sinus (CS) venography. A 37-year-old male presented with congenitally corrected transposition of the great arteries and mesocardia, along with a history of two open heart surgeries (closure of atrial septal defects and a ventricular septal defect, and pulmonary valvectomy at age 7; mechanical tricuspid valve replacement at age 13). He showed symptoms of progressive heart failure (NYHA class III) with significant impairment of the systemic right ventricular function. He also developed permanent atrial fibrillation with a junctional rhythm at a rate of 45 beats per minute. Biventricular pacing without an atrial lead was considered to be the best option available. CRT implantation was facilitated by proper identification of CS anatomy utilizing cardiac CT and CS venography and was performed without any complications. At follow up, a postero-anterior chest X-ray showed the final position of the right-sided ventricular (left ventricular morphology) lead pointing to the apex and the left ventricular lead at the posterolateral aspect of the systemic ventricle (right ventricular morphology).

Objective: We sought to define the inferior sinus venosus defect anatomically and document successful surgical approaches., Methods: We identified all patients previously given a diagnosis of an inferior sinus venosus defect at the Hospital for Sick Children, Toronto, Canada, between 1982 and 2005 by interrogating the cardiology and cardiac surgery databases. We included those having interatrial communications in which 1 or more of the right pulmonary veins drained to the inferior caval vein but retained connection with the left atrium, the rims of the oval fossa, and the walls of the coronary sinus, both being intact., Results: We identified 11 children who had an interatrial communication meeting the criteria for and undergoing surgical repair of an inferior sinus venosus defect. Median age was 1.2 years; 6 (55%) subjects were male, and none were cyanotic. Transthoracic echocardiographic analysis was performed preoperatively in all children, revealing right ventricular dilation in all. Surgical repair was accomplished with a pericardial patch. A complex baffle was needed in 3 children to maintain unobstructed inferior caval and pulmonary venous return. The echocardiographic diagnosis was complete in only 5 patients, but all diagnoses were correct since the year 2000. In all children the observations at surgical intervention showed that the defect was a venoatrial communication involving drainage of the right pulmonary veins to the inferior caval vein while retaining connection to the left atrium., Conclusions: Transthoracic echocardiographic analysis should remain the modality of choice for diagnosis of the inferior sinus venosus defect. We report excellent surgical results with a patch or baffle, correctly redirecting the anomalous venoatrial connections.

Objectives: This study was designed to report a novel indication for percutaneous pulmonary valve implantation in patients with previous right ventricular outflow tract (RVOT) patch., Background: Current indications for percutaneous pulmonary valve implantation are limited to patients who had pulmonary valve stenosis and/or regurgitation in a right ventricle-to-pulmonary artery conduit. Percutaneous pulmonary valve implantation has not been previously reported in patients with severe pulmonary valve regurgitation following repair of tetralogy of Fallot (TOF) using RVOT patch., Methods: After assessment of the RVOT patch in multiple projections, a catheter was placed in a distal pulmonary artery branch. In patients with an RVOT patch, sizing of the narrowest diameter of the RVOT patch by manual inflation of a sizing balloon was performed; a stent was placed into the RVOT patch at the level of the narrowest area to anchor the stent and to create an artificial conduit to place the Melody valve. The percutaneous valve was then implanted., Results: Seven females and 6 males with a mean age of 14.3 years and mean body weight 45 kg had successful percutaneous implantation of the Melody valve. Four patients had previous repair of TOF using RVOT patch. All patients were discharged within 2 days after the procedure without complications. After a mean of 4 months follow-up all patients were alive and well. Transthoracic echocardiography showed competent pulmonary valve. Chest X-ray showed no stent migration or fracture., Conclusions: Percutaneous pulmonary valve implantation can be performed in patients with pulmonary valve regurgitation, including those with previous RVOT patch using pre-stenting techniques, with satisfactory results.