A 52-year-old man presented to an emergency department with a 1-month history of chest tightness and respiratory asphyxia that exacerbated within the last 2 hours. He additionally had a 30-year history of exertional palpitations. Physical examination revealed cyanosis of the lips, irregular, accelerated heart rate, and a systolic murmur in the area of the mitral valve on auscultation. The right and left superior and inferior pulmonary veins converged into the pulmonary vein trunk (Supplementary material, Figure S1). Computed tomography angiography revealed abnormal communication between the pulmonary venous trunk and the superior vena cava, with the pulmonary vein ultimately draining into the right atrium (Figure 1A and 1B). Ultrasonography indicated the presence of an atrial septal defect as well as evidence of a bidirectional shunt between the left and right atrium (Figure 1C and 1D).
Based on these findings, the patient was diagnosed with supracardiac total anomalous pulmonary venous connection (TAPVC). Virtually all cases of TAPVC occur in infants, and the mortality rate by the age of 12 months in untreated cases is 80%.1 Diagnoses of this condition in older adults are extremely rare. In the present case, the left atrium did not receive any direct pulmonary venous return, while the right atrium received systemic venous return, resulting in the right atrial enlargement.2 We speculate that the bidirectional shunt observed between the left and right atrium would compensate for these abnormalities over the course of several decades, and that it may be the key to the survival of patients exhibiting cardiovascular developmental abnormalities.3 This is supported by the color Doppler results and ejection fraction score (69%) for this patient. After his symptoms were alleviated through medical treatment, the patient was discharged home, and he has remained stable on follow-up after 6 months.
Heng Ma, MD, Department of Radiology, Qingdao University Medical College Affiliated Yantai Yuhuangding Hospital, 20 Yudong Road, Yantai 264 000, Shandong Province, China, phone: +86 0535 6691999, email: mhyhdyy@163.com
October 29, 2023.
November 9, 2023.
November 14, 2023.
We are grateful to Wei Yue from Siemens (Jinan, 250000, China) for his assistance with image reconstruction.
This work was supported by Shandong Province Natural Science Foundation (No. ZR2022MH274) and “Taishan Scholar” Project (No. tsqn202103197); to HM.
None declared.
Xia Y, Yang X, Sun Z, et al. A middle-aged man with total anomalous pulmonary venous connection. Pol Arch Intern Med. 2024; 134: 16605. doi:10.20452/pamw.16605
- 1.
- Burroughs JT, Edwards JE. Total anomalous pulmonary venous connection. Am Heart J. 1960; 59: 913-931.Crossref
- 2.
- Rosenquist GC, Kelly JL, Chandra R, et al. Small left atrium and change in contour of the ventricular septum in total anomalous pulmonary venous connection: a morphometric analysis of 22 infant hearts. Am J Cardiol. 1985; 55: 777-782.Crossref
- 3.
- Guo H, Li W, Ba X, et al. Young woman with hemoptysis. Ann Emerg Med. 2021; 78: e13-e14.Crossref