"These patients have to be cared for by cardiologists with a specific expertise in congenital heart disease, in concert with general practitioners," said Dr. Victor Waldmann of European George Pompidou Hospital, in Paris.
"One of the messages is also that some congenital heart diseases may remain undiagnosed and that the understanding and knowledge of these specific ECG patterns can help physicians to suspect these congenital heart diseases, even in adults, to get them into an appropriate care network," he told Reuters Health by email.
The care for adults with congenital heart disease has emerged as a new subspecialty of cardiology, but nonspecialists also need to recognize the diverse cardiac phenotypes encountered in these patients, according to Dr. Waldmann and colleagues.
The authors outline the key ECG findings of the most common or distinctive subtypes of adult congenital heart disease, which covers tetralogy of Fallot, Ebstein anomaly, transposition of the great arteries with intra-atrial baffle repair, congenitally corrected transposition of the great arteries, univentricular hearts and Fontan surgery, atrioventricular septal defect, and atrial septal defect.
Tetralogy of Fallot (TOF) is the most common cyanotic heart defect (found in one in 3,500 to 4,300 adults and representing 7% to 10% of all congenital heart diseases). On ECG, the QRS axis is either normal or has a right deviation attributable to right ventricular hypertrophy and dilatation, and a right bundle branch block is almost universal.
In adults with repaired TOF, QRS prolongation (especially with a QRS duration of 180 ms or more) is the main ECG risk factor for ventricular arrhythmias, the authors note in JAMA Cardiology. Progressive broadening of the QRS over time has been associated with an increased risk of sudden cardiac death, but risk stratification remains complex.
Atrial septal defect accounts for 30% of congenital heart disease detected in adulthood. Right ventricular overload resulting from the defect can manifest as an unusual form of incomplete right bundle branch block, and a notch in the terminal R wave in the inferior leads is a highly specific finding reported in 75% of patients with atrial septal defects, according to the authors.
Late repair of atrial septal defects can be associated with an increased risk of atrial arrhythmia, which is associated with higher overall mortality, especially in men.
"While congenital heart diseases may be diagnosed or suspected by ECG observation in adults with no previous diagnosis, specific markers have also been identified to optimize risk stratification in certain defects, and their periodic surveillance is of clinical relevance," the authors conclude. "The understanding of the main ECG features is of particular importance, not only for adults with congenital heart disease (ACHD) specialists, but also for general cardiologists, since the population of patients with ACHD is continuously expanding."
By Will Boggs MD
SOURCE: https://bit.ly/3jb4XmF JAMA Cardiology, online August 19, 2020.
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