JACC. Case reports · 2026
Congenital Idiopathic Dilatation of the Right Atrium: Prenatal Diagnosis and 9-Year Follow-Up.
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Madrid Marín Ana L, González Nathalia Melo, Quintero Juan C, Madrid Pinilla Antonio J
Аннотация
BACKGROUND: Congenital idiopathic dilatation of the right atrium (CIDRA) is a rare anomaly with scattered reports. Prenatal recognition is challenging given echocardiographic overlap with entities with distinct prognosis and management.
CASE SUMMARY: A fetus had severe right atrial enlargement at 31 1/7 weeks. Massive dilatation with disproportionately mild tricuspid regurgitation established the diagnosis after exclusion of 5 competing entities. Serial surveillance showed progressive enlargement without ventricular dysfunction. Delivery was at term. Through 9 years of conservative management, the patient remained asymptomatic with preserved hemodynamics.
DISCUSSION: This case provides prospective natural history data from fetal life through school age, rarely available. Low-burden supraventricular ectopy at age 9 represents the first detectable electrophysiological change after a long quiescent course and supports continued rhythm surveillance.
TAKE-HOME MESSAGES: Disproportionately mild tricuspid regurgitation relative to the degree of right atrial enlargement is the hallmark echocardiographic discriminator of CIDRA from primary valvular pathology in fetal echocardiography, and its recognition requires systematic exclusion of 5 competing diagnoses with distinct prognostic implications. Long-term conservative management is a valid strategy in asymptomatic patients with CIDRA with preserved hemodynamics; The later appearance of low-burden supraventricular ectopy, although of uncertain prognostic significance on its own, is a reasonable prompt to continue-rather than relax-periodic rhythm surveillance.
CASE SUMMARY: A fetus had severe right atrial enlargement at 31 1/7 weeks. Massive dilatation with disproportionately mild tricuspid regurgitation established the diagnosis after exclusion of 5 competing entities. Serial surveillance showed progressive enlargement without ventricular dysfunction. Delivery was at term. Through 9 years of conservative management, the patient remained asymptomatic with preserved hemodynamics.
DISCUSSION: This case provides prospective natural history data from fetal life through school age, rarely available. Low-burden supraventricular ectopy at age 9 represents the first detectable electrophysiological change after a long quiescent course and supports continued rhythm surveillance.
TAKE-HOME MESSAGES: Disproportionately mild tricuspid regurgitation relative to the degree of right atrial enlargement is the hallmark echocardiographic discriminator of CIDRA from primary valvular pathology in fetal echocardiography, and its recognition requires systematic exclusion of 5 competing diagnoses with distinct prognostic implications. Long-term conservative management is a valid strategy in asymptomatic patients with CIDRA with preserved hemodynamics; The later appearance of low-burden supraventricular ectopy, although of uncertain prognostic significance on its own, is a reasonable prompt to continue-rather than relax-periodic rhythm surveillance.