Grown-up congenital heart disease

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Total cavopulmonary connection, commonly referred to as the Fontan procedure, is the established destination therapy for univentricular hearts. While the procedure permits a longer survival of these patients, this circulation involves several compromises from normal human circulation and poses several challenges with increasing age after surgery. We present an instance of acute ST-elevation myocardial infarction with Guillain-Barre syndrome in an adult after Fontan palliation and discuss the challenges in management...

Progress towards the development and adoption of minimally invasive techniques in cardiac surgery has been slower than that seen in other surgical specialties. Congenital heart disease (CHD) patients represent an important population within cardiac disease, of which atrial septal defect (ASD) is one of the most common diagnoses. Management of ASD encompasses a range of minimal-access and minimally invasive approaches, including transcatheter device closure, mini-sternotomy, thoracotomy, video-assisted, endoscopic, and robotic approaches...

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Background Prolonged post-surgery intensive care unit (ICU) stay for congenital heart disease (CHD) has been explored in the pediatric population. However, there is limited data for adult CHD (ACHD), also called grown-up congenital heart (GUCH) disease, especially in low-resource countries where intensive care beds are scarce. This study identifies factors associated with prolonged ICU stay following surgery for ACHD in Pakistan, a lower-middle-income country (LMIC). Methods This retrospective study included all adult patients (⩾18 years) who underwent cardiac surgery with cardiopulmonary bypass for their CHD from 2011-2016 at a tertiary-care private hospital in Pakistan...

PURPOSE: To explore the relationships between growth trajectory, parenting stress and parent post-traumatic stress (PTS), in infants with congenital heart disease, and the moderating role of parents' dyadic adjustment on those associations. DESIGN AND METHODS: A secondary analysis of data from the REACH Telehalth home monitoring multi-site randomized clinical trial. Parents completed the Parenting Stress Index (PSI), Post-traumatic diagnostic scale, and the Dyadic Adjustment Scale...

Inadvertent placement of pacemaker and implantable cardioverter-defibrillator (ICD) leads in the left ventricle is a rare but well-recognized complication of device implantation. We report a case of an unicameral ICD lead inadvertently placed through the left subclavian artery right positioned, across the aortic valve into the left ventricle, in a patient with situs viscerum inversus. A transthoracic echocardiogram about a month after the procedure showed an unusual course of the lead. The lead was successfully removed without complications or sequelae...

BACKGROUND: The belief that genetics plays a major role in the pathogenesis of congenital heart defects (CHD) has grown popular among clinicians. Although some studies have focused on the genetic testing of foetuses with CHD in China, the genotype-phenotype relationship has not yet been fully established, and hotspot copy number variations (CNVs) related to CHD in the Chinese population are still unclear. This cohort study aimed to assess the prevalence of chromosomal abnormalities in Chinese foetuses with different types of CHD...

OBJECTIVES:  Patients with repaired complete atrioventricular septal defect (CAVSD) represent an increasing portion of grown-ups with congenital heart disease. For repair of CAVSD, the single-patch technique has been employed first. This technique requires division of the bridging leaflets, thus, among other issues, long-term function of the atrioventricular valves is of particular concern. METHODS:  Between 1978 and 2001, 100 consecutive patients with isolated CAVSD underwent single-patch repair in our institution...

Sustained re-entrant tachyarrhythmias treatment has become pivotal in the grown-up congenital heart patients clinical management. Cardiac LGE-MRI tissue characterization integrated with high definition electroanatomic map could allow fast recognition and effective treatment of substrate of tachyarrhythmias. Cardiac LGE-MRI areas were suggestive of post-surgical changes both in atrium and ventricle. High-density electro-anatomical map localized areas of slow conduction identifying conducting isthmuses of re-entrant arrhythmias...

This case report describes a successful leadless pacemaker implant (Micra VR Medtronic, Inc, Minneapolis, MN) in a 48-year-old patient with a history of Mustard repair. Twenty-one years after dual-chamber pacemaker implant, both conventional leads became dysfunctional. Lead extraction was refused by the patient and the subclavian vein was obstructed. A leadless pacemaker was selected as an alternative. Intracardiac echocardiography allowed the safe introduction of the delivery system into the non-systemic left ventricle...

Background: COVID-19 pandemic has disrupted pediatric cardiac services across the globe. Limited data are available on the impact of COVID.19 on pediatric cardiac care in India. Aims: The aims are to study the impact of COVID-19 pandemic on the care of children with heart disease in India in terms of number of outpatient visits, hospitalizations, catheter-based interventions, and cardiac surgeries. Settings and Design: This is a retrospective, multicentric, observational study...

Professor Liesl Zühlke is the focus of our fifth in a series of interviews in Cardiology in the Young entitled, "Global Leadership in Paediatric and Congenital Cardiac Care". Professor Zühlke (nee Hendricks) was born in Cape Town, South Africa. She would attend medical school in her hometown at University of Cape Town, graduating in 1991. Professor Zühlke then went on to complete a Diploma in Child Health at College of Medicine in Cape Town followed by completion of her Paediatric and Paediatric Cardiology training in 1999 and 2007, respectively...

Identifying adults with congenital heart disease (CHD) at high risk for sudden death who stand to benefit from primary prevention implantable cardioverter-defibrillators (ICDs) remains a major challenge. Inconsistencies among evidence-based recommendations by various professional societies can be a source of confusion to practicing clinicians. This article summarizes and compares recommendations from the Canadian Cardiovascular Society management guidelines on adults with CHD (2009), expert consensus from the Pediatric and Congenital Electrophysiology Society and Heart Rhythm Society (2014), European Society of Cardiology (ESC) guidelines on sudden death (2015), position statement from the European Heart Rhythm Association, Association for European Paediatric and Congenital Cardiology, and ESC working group on grown-up CHD (2018), and ESC guidelines on adult CHD (2020)...

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 : <AbstractText Label="OBJECTIVES" NlmCategory="OBJECTIVE">We sought to evaluate the predictive power of the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STAT) mortality score and the adult congenital heart surgery (ACHS) mortality score for the adults undergoing congenital heart operations entered into the European Congenital Heart Surgeons Association (ECHSA) database. METHODS: The data set comprised 17 662 major operations performed between 1997 and 2019, on patients 18 years of age or older, in European centres participating in the ECHSA database...

OBJECTIVES: Machine learning methods potentially provide a highly accurate and detailed assessment of expected individual patient risk before elective cardiac surgery. Correct anticipation of this risk allows for the improved counselling of patients and avoidance of possible complications. We therefore investigated the benefit of modern machine learning methods in personalized risk prediction for patients undergoing elective heart valve surgery. METHODS: We performed a monocentric retrospective study in patients who underwent elective heart valve surgery between 1 January 2008 and 31 December 2014 at our centre...

In this review we provide a brief description of recently published articles addressing topics relevant to pediatric cardiologists. Our hope is to provide a summary of the latest articles published recently in other journals in our field. The articles address (1) The impact of COVID-19 in individuals with congenital heart disease through the life span. Patients with a genetic syndrome and adults at advanced physiological stage were at highest risk for moderate/severe infection. (2) Echocardiographic findings of the multisystem inflammatory syndrome in children showing a high prevalence of myocardial injury and systolic dysfunction that improves in the subacute phase...

Background: Our historical overview in the form of a cohort study aimed to describe the changes in the cardiac operations in adults with congenital heart disease (ACHD), over 45 years in the German Heart Centre in Munich. Methods: The study population comprised all consecutive patients aged 18 or more, who underwent surgery for congenital heart disease (CHD) at the German Heart Centre Munich, between 1974 and 2018. Operations were coded according to the Adult Congenital Heart Surgery (ACHS) score...

BACKGROUND: Reported prevalence of congenital heart diseases (CHDs) varies widely among studies worldwide. The incidence of CHD, total number of pediatric and adult grown-up congenital heart disease (GUCH), is not determined in Iran. Therefore, we have designed a system to register the information of patients with CHD for the first time in our country. METHODS: CHD registry is a database in which the patients' data are collected by five pediatric cardiologists from three referral hospitals affiliated to Isfahan University of Medical Sciences, Isfahan, Iran, and five outpatient clinics...