Effect of Sodium-Glucose Cotransporter 2 Inhibitors in Adults With Congenital Heart Disease.

Background: Heart failure (HF) is the principal cause of morbidity and mortality in adults with congenital heart disease (ACHD). Robust evidence-based treatment options are lacking.

Objectives: This study aims to evaluate the safety, tolerability, and short-term HF-related effects of sodium-glucose cotransporter 2 inhibitors (SGLT2i) in a real-world ACHD population.

Percutaneous heart valves demonstrating long-term durability: A case series of Melody valves in the pulmonary position lasting up to 19 years.

It is uncertain how long catheter delivered percutaneous heart valves may last. In congenital cardiology, stenosis and regurgitation of right ventricular to pulmonary artery conduits and valves is common, leading to repeated operations for young patients with concomitant mortality and morbidity. It has also been unclear whether percutaneous pulmonary valves last as long as surgical pulmonary valves.

Echocardiographic Screening for Pulmonary Hypertension in Congenital Heart Disease: JACC Review Topic of the Week.

Echocardiography is the mainstay in screening for pulmonary hypertension (PH). International guidelines suggest echocardiographic parameters for suspecting PH, but these may not apply to many adults with congenital heart disease (ACHD). PH is relatively common in ACHD patients and can significantly affect their exercise capacity, quality of life, and prognosis.

Occlutech percutaneous patent foramen ovale closure: Safety and efficacy registry (OPPOSE).

Introduction: Safety and efficacy data on patent foramen ovale (PFO) closure with the Occlutech Figulla Flex II device are lacking. We undertook a fully monitored prospective Registry on PFO closure using this device.

Methods: 100 patients undergoing PFO closure were enrolled into the OPPOSE Registry at 6 UK centres.

Initial use of the new GORE(®) septal occluder in patent foramen ovale closure: implantation and preliminary results.

Background: A number of devices are available for percutaneous closure of a clinically significant patent foramen ovale (PFO). The new GORE(®) septal occluder (GSO) is a nonself-centering device consisting of an expanded polytetrafluoroethylene tube supported by a frame of nitinol wire conforming into a double disk. This study reports the first clinical GSO implantation experience.

Over-the-wire-technique device implantation.

Introduction: Transcatheter closure of cardiac defects is a mainstay of treatment in congenital and structural heart disease. Occasionally, the devices used are useful in nonstandard or difficult positions but device embolization and malposition can be complicating factors necessitating percutaneous retrieval or emergency surgery. We describe a new "over-the-wire" technique that allows guided safe deployment and easy retrievability if required.

A cell-autonomous role of Cited2 in controlling myocardial and coronary vascular development.

Aims: Myocardial development is dependent on concomitant growth of cardiomyocytes and a supporting vascular network. The coupling of myocardial and coronary vascular development is partly mediated by vascular endothelial growth factor (VEGFA) signalling and additional unknown mechanisms. We examined the cardiomyocyte specific role of the transcriptional co-activator Cited2 on myocardial microstructure and vessel growth, in relation to Vegfa expression.

Telescopic catheter-in-long sheath and parallel to a stiff guide wire technique for complex pulmonary artery anatomy.

Selective catheterization and procedures in pulmonary arteries may be very challenging. We developed a technique in which an extra-stiff guide wire was placed in a pulmonary artery. Across it a long sheath was placed proximally or controlateral to the lesion to be treated.

Protein-losing enteropathy resolved by percutaneous intervention.

The extracardiac total cavopulmonary connection is the final stage of palliation of hearts with single-ventricle physiology. Protein-losing enteropathy (PLE) and plastic bronchitis are catastrophic sequale that may occur in patients with the total cavopulmonary connection and may complicate the early and long-term follow-up. Here we report on the successful treatment of a 16-year boy affected by PLE by percutaneous closure of a persistent anterograde pulmonary blood flow by using an 8-mm Amplatzer VSD Occluder.

Initial experience with the Amplatzer Vascular Plug IV in congenital heart disease: coronary artery fistula and aortopulmonary collateral artery embolization.

Background: A number of percutaneous devices are available to embolize anomalous vessels in congenital heart disease. We report our initial single-center experience with the new Amplatzer Vascular Plug IV (AVP IV) in congenital heart disease to embolize a coronary artery fistula and aortopulmonary collateral arteries in 4 cases.

Methods: From August 2009 until April 2010, 7 AVP IV devices were deployed in 4 patients, age range 5 months to 9 years, weight 3.

Managing adults with congenital heart disease in the catheterization laboratory: state of the art.

There has been a rapid growth in interventional cardiology techniques to treat adults with congenital heart disease, mirroring the rise of interventional cardiology as a cardiology subspecialty and the increasing population of adults with congenital abnormalities. Starting with neonatal balloon atrial septostomy for transposition of the great arteries in the 1960s, improving and changing percutaneous interventional techniques have increasingly replaced surgery as a treatment option in several congenital heart diseases. Many pediatric cardiac patients now survive to adulthood following early surgery, perhaps making additional surgery higher risk, and percutaneous procedures offer an alternative approach, although with a different inherent set of risks and benefits in terms of morbidity and mortality.

Aortic coarctation complicated by wall aneurysm: the role of covered stents.

Objectives: To evaluate the management of aneurysms associated with aortic coarctation by covered stent deployment.

Background: Aortic aneurysm formation complicating aortic coarctation carries a risk of rupture with high mortality rate. Surgical repair has a significant mortality and may be associated with central nervous system injury, including paraplegia.

The relationship of systemic right ventricular function to ECG parameters and NT-proBNP levels in adults with transposition of the great arteries late after Senning or Mustard surgery.

Aims: Heart failure is common late after Senning or Mustard palliation of transposition of the great arteries (TGA). Although cardiac magnetic resonance (CMR) is the gold standard for evaluating systemic right ventricular performance, additional information regarding heart failure status might be gleaned from the surface ECG and circulating N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. The interrelationships between these heart failure markers were examined in adults late after Mustard and Senning surgery.

Intracardiac echocardiography off piste? Closure of the left atrial appendage using ICE and local anesthesia.

Left atrial appendage (LAA) occlusion is increasingly accepted to reduce the risk of stroke in patients with atrial arrhythmia who are unsuitable for routine anticoagulation. It is generally performed under general anesthesia, guided by transoesophageal echocardiography with accurate imaging being essential for correct deployment of the device. We present a case where LAA occlusion was done under local anesthesia in a high-anesthetic risk patient, using novel placement of an intracardiac echo probe via a Mullins sheath in the right ventricular outflow tract and pulmonary artery.

Epiblastic Cited2 deficiency results in cardiac phenotypic heterogeneity and provides a mechanism for haploinsufficiency.

Aims: Deletion of the transcription factor Cited2 causes penetrant and phenotypically heterogenous cardiovascular and laterality defects and adrenal agenesis. Heterozygous human CITED2 mutation is associated with congenital heart disease, suggesting haploinsufficiency. Cited2 functions partly via a Nodal-->Pitx2c pathway controlling left-right patterning.

Molecular mechanisms controlling the coupled development of myocardium and coronary vasculature.

Cardiac failure affects 1.5% of the adult population and is predominantly caused by myocardial dysfunction secondary to coronary vascular insufficiency. Current therapeutic strategies improve prognosis only modestly, as the primary cause -- loss of normally functioning cardiac myocytes -- is not being corrected.

Intravascular ultrasound to guide stenting of an anomalous right coronary artery coursing between the aorta and pulmonary artery.

We report on a case of anomalous right coronary artery with interarterial course which caused severe symptoms and was treated percutaneously after failed bypass surgery. Using intravascular ultrasound (IVUS) guidance, a drug eluting stent was placed at the ostium of the anomalous artery, correcting both the baseline narrowing and the phasic, systolic arterial compression.

Left ventricular pseudoaneurysm and rupture after limited myocardial infarction.

A 50-year-old man had a large lateral left ventricular wall pseudoaneurysm with rupture after a limited myocardial infarction. This was diagnosed by the combination of flow in and out of the aneurysm and pulsatile flow in the pericardial cavity using color flow and continuous wave Doppler ultrasound. This report describes the case management, the use of echocardiography in this setting, and the implications of pseudoaneurysm formation.