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Risk Factors for Increased Hospital Resource Utilization and In-Hospital Mortality in Adults With Single Ventricle Congenital Heart Disease.

Am J Cardiol. 2016 Aug 01;118(3):453-62

Authors: Collins RT, Doshi P, Onukwube J, Fram RY, Robbins JM

Most patients with single ventricle congenital heart disease are now expected to survive to adulthood. Co-morbid medical conditions (CMCs) are common. We sought to identify risk factors for increased hospital resource utilization and in-hospital mortality in adults with single ventricle. We analyzed data from the 2001 to 2011 Nationwide Inpatient Sample database in patients aged ≥18 years admitted to nonteaching general hospitals (NTGHs), TGHs, and pediatric hospitals (PHs) with either hypoplastic left heart syndrome, tricuspid atresia or common ventricle. National estimates of hospitalizations were calculated. Elixhauser CMCs were identified. Length of stay (LOS), total hospital costs, and effect of CMCs were determined. Age was greater in NTGH (41.5 ± 1.3 years) than in TGH (32.8 ± 0.5) and PH (25.0 ± 0.6; p <0.0001). Adjusted LOS was shorter in NTGH (5.6 days) than in PH (9.7 days; p <0.0001). Adjusted costs were higher in PH ($56,671) than in TGH ($31,934) and NTGH ($18,255; p <0.0001). CMCs are associated with increased LOS (p <0.0001) and costs (p <0.0001). Risk factors for in-hospital mortality included increasing age (odds ratio [OR] 5.250, CI 2.825 to 9.758 for 45- to 64-year old vs 18- to 30-year old), male gender (OR 2.72, CI 1.804 to 4.103]), and the presence of CMC (OR 4.55, CI 2.193 to 9.436) for 2 vs none). No differences in mortality were found among NTGH, TGH, and PH. Cardiovascular procedures were more common in PH hospitalizations and were associated with higher costs and LOS. CMCs increase costs and mortality. In-hospital mortality is increased with age, male gender, and the presence of hypoplastic left heart syndrome.

PMID: 27291967 [PubMed - indexed for MEDLINE]

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Demographics and Epidemiology of Sudden Deaths in Young Competitive Athletes: From the United States National Registry.

Am J Med. 2016 Nov;129(11):1170-1177

Authors: Maron BJ, Haas TS, Ahluwalia A, Murphy CJ, Garberich RF

BACKGROUND: Sudden deaths in young competitive athletes are tragic events, with high public visibility. The importance of race and gender with respect to sport and the diagnosis and causes of sudden death in athletes has generated substantial interest.
METHODS: The US National Registry of Sudden Death in Athletes, 1980-2011, was accessed to define the epidemiology and causes of sudden deaths in competitive athletes. A total of 2406 deaths were identified in young athletes aged 19 ± 6 years engaged in 29 diverse sports.
RESULTS: Among the 842 athletes with autopsy-confirmed cardiovascular diagnoses, the incidence in males exceeded that in females by 6.5-fold (1:121; 691 vs 1:787,392 athlete-years; P ≤.001). Hypertrophic cardiomyopathy was the single most common cause of sudden death, occurring in 302 of 842 athletes (36%) and accounting for 39% of male sudden deaths, almost 4-fold more common than among females (11%; P ≤.001). More frequent among females were congenital coronary artery anomalies (33% vs 17% of males; P ≤.001), arrhythmogenic right ventricular cardiomyopathy (13% vs 4%; P = .002), and clinically diagnosed long QT syndrome (7% vs 1.5%; P ≤.002). The cardiovascular death rate among African Americans/other minorities exceeded whites by almost 5-fold (1:12,778 vs 1:60; 746 athlete-years; P <.001), and hypertrophic cardiomyopathy was more common among African Americans/other minorities (42%) than in whites (31%; P ≤.001). Male and female basketball players were 3-fold more likely to be African American/other minorities than white.
CONCLUSIONS: Within this large forensic registry of competitive athletes, cardiovascular sudden deaths due to genetic and/or congenital heart diseases were uncommon in females and more common in African Americans/other minorities than in whites. Hypertrophic cardiomyopathy is an under-appreciated cause of sudden death in male minority athletes.

PMID: 27039955 [PubMed - indexed for MEDLINE]

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An adult case of Kommerell's diverticulum with a right-sided aortic arch.

Asian Cardiovasc Thorac Ann. 2017 Jan 01;:218492317707146

Authors: Tsutsumi K, Nishida K, Tsukamoto J, Iijima N, Ishida O

The patient was a 56-year-old woman with Kommerell's diverticulum associated with a right-sided aortic arch with mirror-image branching. No other congenital heart anomalies or vascular rings were observed. Descending aortic replacement through a right posterolateral thoracotomy was performed to eliminate the risk of diverticular rupture. The patient's postoperative course was uneventful. This was a rare adult case of right-sided aortic arch with Kommerell's diverticulum associated with no other congenital heart disease.

PMID: 28457170 [PubMed - as supplied by publisher]

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Introduction to the Congenital Heart Defects: Anatomy of the Conduction System.

Card Electrophysiol Clin. 2017 Jun;9(2):167-175

Authors: Moore JP, Aboulhosn JA

The position and course of the conduction system in congenital heart disease are intricately tied to the underlying congenital malformation. Although only subtle differences exist between the anatomy of the conduction axis for simple congenital heart lesions and normal anatomy, almost every patient with congenital heart disease harbors some important anatomic variation. This article summarizes the body of literature by retaining original classical concepts and by attempting to translate the available knowledge into useful points for the congenital heart disease specialist. This discussion spans the entire spectrum of simple to complex congenital heart disease.

PMID: 28457233 [PubMed - in process]

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Bradyarrhythmias in Congenital Heart Disease.

Card Electrophysiol Clin. 2017 Jun;9(2):177-187

Authors: Carlson SK, Patel AR, Chang PM

Bradyarrhythmias in adults with congenital heart disease (CHD) comprise a complex group of arrhythmia disorders with congenital and acquired origins, highly variable long-term sequelae, and complicated treatment options. They can develop across the spectrum of CHD defects and can be encountered at all ages. Although permanent pacing is effective in treating bradyarrhythmias, it is associated with many complications and morbidity, where it is often used early in life. This section discusses the incidence and prevalence of bradyarrhythmias in the CHD population, their timing of occurrence with respect to specific disease entities and interventions, and their short- and long-term clinical sequelae.

PMID: 28457234 [PubMed - in process]

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Supraventricular Tachycardia in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Card Electrophysiol Clin. 2017 Jun;9(2):189-211

Authors: Janson CM, Shah MJ

Supraventricular arrhythmias represent a major source of morbidity in adults with congenital heart disease (ACHD). Anatomic variants and post-operative changes contribute to a unique electrophysiologic milieu ripe for the development of supraventricular tachycardia. Intra-atrial reentrant tachycardia is the most prevalent mechanism. Atrioventricular reciprocating tachycardia is common in lesions associated with accessory pathways. Abnormal anatomy complicates the management of atrioventricular nodal reentrant tachycardia. Tachycardia mediated by twin atrioventricular nodes is rare. Focal tachycardias are considerations in the ACHD population. Each of these tachycardia mechanisms is reviewed, focusing on the inherent diagnostic and therapeutic challenges.

PMID: 28457235 [PubMed - in process]

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Ventricular Arrhythmias in Adult Congenital Heart Disease: Mechanisms, Diagnosis, and Clinical Aspects.

Card Electrophysiol Clin. 2017 Jun;9(2):213-223

Authors: Sathananthan G, Harris L, Nair K

The risk of ventricular arrhythmias in the adult congenital heart disease population increases with age. The mechanism, type, and frequency vary depending on the complexity of the defect, whether it has been repaired, and the type and timing of repair. Risk stratification for sudden death in patients with congenital heart disease is often challenging. Current recommendations provide a useful guide for management of these patients and risk stratification continues to evolve. Internal cardiac defibrillator implantation is often challenging due to limited transvenous access, often resulting in the need for epicardial or subcutaneous devices.

PMID: 28457236 [PubMed - in process]

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Sudden Cardiac Death in Adult Congenital Heart Disease.

Card Electrophysiol Clin. 2017 Jun;9(2):225-234

Authors: Ávila P, Chaix MA, Mondésert B, Khairy P

Sudden death of presumed arrhythmic etiology is a leading cause of mortality in adults with congenital heart disease. Anticipated benefits of the implantable cardioverter-defibrillator (ICD) must be weighed against high complication rates. Without robust evidence from randomized trials, caregivers face difficult decisions in selecting appropriate candidates. Although secondary prevention indications are often clear-cut, risk stratification for primary prevention ICDs is more challenging. Factors associated with sudden death in patients with tetralogy of Fallot are reasonably consistent across studies. In contrast, identification of high-risk patients with systemic right ventricles or univentricular hearts remains controversial.

PMID: 28457237 [PubMed - in process]

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Drug Therapy in Adult Congenital Heart Disease.

Card Electrophysiol Clin. 2017 Jun;9(2):295-309

Authors: Contractor T, Levin V, Mandapati R

Adults with congenital heart disease are at risk for atrial and ventricular arrhythmias that can lead to an increased morbidity as well as mortality. When catheter ablation is not an option or unsuccessful, antiarrhythmic drugs are the mainstay of treatment. There is limited data on the use of antiarrhythmics in this population. The purpose of this article is to discuss the practical aspects of the use of antiarrhythmics in adults with congenital heart disease. Several tables have been provided to provide clinicians a reference for daily use.

PMID: 28457243 [PubMed - in process]

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Cardiac Arrhythmias in Adults with Congenital Heart Disease: Pacemakers, Implantable Cardiac Defibrillators, and Cardiac Resynchronization Therapy Devices.

Card Electrophysiol Clin. 2017 Jun;9(2):319-328

Authors: Cecchin F, Halpern DG

Implanting cardiac rhythm medical devices in adults with congenital heart disease requires training in congenital heart disease. The techniques and indications for device implantation are specific to the anatomic diagnosis and state of disease progression. It often requires a team of physicians and is best performed at a specialized adult congenital heart center.

PMID: 28457245 [PubMed - in process]