Wheezing as a sign of cor triatriatum sinister culminating in multiple organ failure

Abstract

Cardiac asthma or cardiac wheezing (CW) refers to a syndrome of dyspnea and wheezing that mimicks asthma clinically. Reported herein is the case of a 2-month-old boy who presented with refractory wheezing as a sign of cor triatriatum sinister (CTS) that culminated in overwhelming multiple organ failure in a short time. On the day of admission, oxygen saturation (SpO2 ) was <80%. Heart rate was 198 beats/min and respiratory rate 58 breaths/min. Chest radiogram showed pulmonary edema. Electrocardiogram showed right atrial enlargement and right ventricular hypertrophy. N-terminal pro-brain natriuretic peptide (NTproBNP) was very high at >20,000 pg/mL. Two-dimensional echocardiography with Doppler showed CTS, which was complicated with severe pulmonary arterial hypertension due to flagrant pulmonary venous obstruction. Cardiac surgery was undertaken, after which pulmonary edema subsided, SpO2 increased to ≥96%, and NTproBNP dropped to normal. He was discharged 11 days later, and was free of cardiac, pulmonary, renal, and neurological sequelae at 24 month follow up.

Cor Triatriatum: Case Report of Emergency Department Diagnosis

“…Misdiagnosis of cor triatriatum as reactive airway disease or asthma has been reported occasionally. 2 , 3 , 9 , 10 Even so, the diagnosis remains elusive and challenging particularly in the ED. Given the prevalence of asthma and other reactive airway disorders in childhood, with up to 11% of all children aged 10 years having episodes of reactive airway disease, 11 it is unsurprising that a presentation as in the case above would prompt initial ED management with bronchodilators, inhaled beta-agonists, and corticosteroids.…”

Cor Triatriatum: A Review

“…In neonates, the characteristic features of neonatal respiratory distress syndrome and cardiac asthma may arise, leading to endotracheal intubation and mechanical ventilation till obstruction is relieved. 4,38 Degenerative changes may take place in the abnormal septum, especially in adults and older age groups, leading to fibrosis and calcification very similar to rheumatic changes as seen in the mitral valve. [39][40][41][42] Thus, gradual narrowing of membrane orifice manifests symptomatically in the advanced age groups.…”