Arrhythmias in Adult Patients With Congenital Heart Disease

Walsh, Edward P.; Cecchin, Frank

doi:10.1161/circulationaha.105.592410

Cited by 366 publications

(264 citation statements)

References 124 publications

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“…This can be present due to sinus node dysfunction in patients with left atrial isomerism, a laterality disorder characterized by bilateral left sidedness. 9,10 Here, we describe a large four-generation family with an autosomal dominant condition, comprising various types of CHD and low atrial rhythm, a unique phenotype not described before within a family. A genome-wide linkage analysis yielded linkage to a region of 39 Mb on chromosome 9q with a maximum LOD score of 4.1.…”

Section: Introductionmentioning

confidence: 93%

“…They can be isolated and idiopathic, especially in children, but can also co-occur with CHD. 9 A P-wave frontal axis oriented in a superior direction, reflecting atrial pacemaker tissue located in the lower part of the atrium, is referred to as low atrial rhythm. This can be present due to sinus node dysfunction in patients with left atrial isomerism, a laterality disorder characterized by bilateral left sidedness.…”

Section: Introductionmentioning

confidence: 99%

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A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q

et al. 2011

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Congenital heart defects (CHDs) occur mostly sporadic, but familial CHD cases have been reported. Mutations in several genes, including NKX2.5, GATA4 and NOTCH1, were identified in families and patients with CHD, but the mechanisms underlying CHD are largely unknown. We performed genome-wide linkage analysis in a large four-generation family with autosomal dominant CHD (including atrial septal defect type I and II, tetralogy of Fallot and persistent left superior vena cava) and low atrial rhythm, a unique phenotype that has not been described before. We obtained phenotypic information including electrocardiography, echocardiography and DNA of 23 family members. Genome-wide linkage analysis on 12 affected, 5 unaffected individuals and 1 obligate carrier demonstrated significant linkage only to chromosome 9q21-33 with a multipoint maximum LOD score of 4.1 at marker D9S1690, between markers D9S167 and D9S1682. This 48-cM critical interval corresponds to 39 Mb and contains 402 genes. Sequence analysis of nine candidate genes in this region (INVS, TMOD1, TGFBR1, KLF4, IPPK, BARX1, PTCH1, MEGF9 and S1PR3) revealed no mutations, nor were genomic imbalances detected using array comparative genomic hybridization. In conclusion, we describe a large family with CHD and low atrial rhythm with a significant LOD score to chromosome 9q. The phenotype is representative of a mild form of left atrial isomerism or a developmental defect of the sinus node and surrounding tissue. Because the mechanisms underlying CHD are largely unknown, this study represents an important step towards the discovery of genes implied in cardiogenesis.

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Section: Introductionmentioning

confidence: 93%

Section: Introductionmentioning

confidence: 99%

A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q

et al. 2011

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“…The underlying cause is thought to be multifactorial, including structural abnormalities, longstanding abnormal cardiac hemodynamics, and fibrosis from corrective surgeries. 2 Our patient has no history of CHD, no signs of structural abnormalities on echocardiography, and no history of cardiac surgery.…”

Section: At This Point In the Evaluationmentioning

confidence: 81%

74-Year-Old Man With Palpitations

Zawadowski

Lapeyre

2012

Mayo Clinic Proceedings

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A 74-year-old man with a history of hypertension, hyperlipidemia, and obstructive sleep apnea presented to his primary care physician for his annual examination. His only symptom was palpitations that started 6 months previously. The palpitations had been occurring several times per day, lasting only a few seconds on each occasion. They were not associated with activity or any particular time of the day. He had no other associated symptoms, including dyspnea, angina, or syncope. He reported no alcohol use and drank one cup of coffee daily. His medications included aspirin, losartan, hydrochlorothiazide, and simvastatin.On examination, the patient was afebrile. His blood pressure was 142/70 mm Hg, and his pulse rate was 60 beats/min. Cardiovascular examination revealed a nondisplaced point of maximal impulse and irregular rhythm with frequent premature beats. Heart sounds were normal, no murmurs were heard, and jugular venous pressure was not elevated. The lungs were clear on auscultation bilaterally. His abdomen was soft and not distended, and no peripheral edema was noted.Laboratory studies yielded the following results (reference ranges shown parenthetically): hemoglobin, 14.4 g/dL (13.5-17.5 g/dL); leukocytes, 7.0 ϫ

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“…[1][2][3] It is associated with a higher risk of heart failure, ventricular dysfunction, and thromboembolic complications. [1][2][3][4] Restoration and maintenance of the sinus rhythm is therefore important to prevent these complications. 5 There are limited data on the outcomes of elective direct current cardioversion (DCCV) in adults with CHD.…”

mentioning

confidence: 99%

Outcomes of Direct Current Cardioversion in Adults With Congenital Heart Disease

Egbe

Asirvatham

Kapa

et al. 2017

Journal of the American College of Cardiology

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Few data exist on direct current cardioversion (DCCV) in adult patients with congenital heart disease (CHD). This is a retrospective case-control study of 279 adults with CHD and 279 adults without CHD (control group) who had elective DCCV for atrial arrhythmias at Mayo Clinic, 2001 to 2013. Control patients were matched by gender and arrhythmia type. The objective was to compare DCCV procedural failure (failure to terminate the presenting arrhythmia) and arrhythmia recurrence (AR). In the CHD group (mean age 55 -20 years; men 166 [59%]), the most common diagnosis was Fontan palliation (61; 22%). Trans-esophageal echocardiography was performed before DCCV in 216 patients (77%); 162 (58%) had atrial flutter, and 117 (42%) had atrial fibrillation. Procedural failure and AR between the case and the control groups were more common in the CHD group (14% vs 7%, p = 0.01) and (83% vs 66% at 60 months, p = 0.001) respectively. There were no deaths or thromboembolic complications. The multivariable risk factors for procedural failure were Fontan palliation and spontaneous echocardiographic contrast; the risk factors for AR were Fontan palliation and atrial fibrillation. When patients with Fontan palliation were excluded from the analysis, the outcome of DCCV (failure and recurrence rates) was similar for the CHD and non-CHD groups despite the age difference between the cohorts. In conclusion, the present study showed that DCCV outcomes were similar for CHD and non-CHD patients, with the exception of patients with Fontan palliation.Atrial arrhythmia is common in adult patients with congenital heart disease (CHD), and the prevalence varies with lesion complexity and age of the patient. [1][2][3] It is associated with a higher risk of heart failure, ventricular dysfunction, and thromboembolic complications. [1][2][3][4] Restoration and maintenance of the sinus rhythm is therefore important to prevent these complications. 5 There are limited data on the outcomes of elective direct current cardioversion (DCCV) in adults with CHD. 6,7 A previous study 6 from our institution, involving a small cohort with limited follow-up, showed that DCCV is safe and effective in terminating atrial arrhythmia in this population. The present study involved a large cohort

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Arrhythmias in Adult Patients With Congenital Heart Disease

Cited by 366 publications

References 124 publications

A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q

A novel autosomal dominant condition consisting of congenital heart defects and low atrial rhythm maps to chromosome 9q

74-Year-Old Man With Palpitations

Outcomes of Direct Current Cardioversion in Adults With Congenital Heart Disease

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