Total anomalous pulmonary vein drainage in a 60-year-old woman diagnosed in an ECG-gated multidetector computed tomography – a case report and review of literature

Czekajska−Chehab, Elżbieta; Tomaszewski, A.; Świętochowska, Elżbieta; Zakościelna, Magdalena; Wojtkowska, Agnieszka; Siek, E.; Staśkiewicz, Grzegorz; Drop, Andrzej

doi:10.5114/pjr.2018.77696

Cited by 4 publications

(3 citation statements)

References 22 publications

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“…Patients with TAPVC have a wide range of clinical manifestations because of the vast differences in anatomy and hemodynamics, ranging from asymptomatic to severe hypoxemia; thus, TAPVC is most easily misdiagnosed as a larger, secondary ASD ( 15 ). These patients present with gradual symptoms, such as shortness of breath, atypical chest pain, or atrial arrhythmia ( 15 - 17 ). However, most TAPVC cases were diagnosed by echocardiography during the initial visit or during physical examination ( 18 ).…”

Section: Discussionmentioning

confidence: 99%

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults

Chen¹,

Gu²,

Han³

et al. 2023

J Thorac Dis

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Background Patients with total anomalous pulmonary venous connection (TAPVC) generally have symptoms during the neonatal period and infancy, and the fatality rate is extremely high. Most patients do not survive to adulthood. This study analyzed the clinical and transthoracic echocardiographic (TTE) manifestations of adult patients with TAPVC, summarized the echocardiographic characteristics of TAPVC, and identified the factors influencing pulmonary hypertension. Methods Data from adult patients with TAPVC from Beijing Anzhen Hospital, China, were retrospectively collected for analyses, including sex, age, history of gestation, clinical manifestations, echocardiographic parameters, and blood oxygen levels. Patients were grouped for comparative analyses based on their pulmonary artery systolic pressure (PASP) (≥60 vs . <60 mmHg); 32 atrial septal defect (ASD) patients were included as a control group. Results (I) Sixteen patients were identified with TAPVC (11 women and 5 men; mean age: 32.2±9.5 years), including 8, 4, and 4 patients with supra-cardiac, mixed, and intracardiac type TAPVC, respectively. Furthermore, 10 patients had moderate or severe tricuspid regurgitation, and 6 had a PASP of ≥60 mmHg. Echocardiography misdiagnosed 2 patients with an ASD. (II) The TAPVC group patients had a smaller left atrium (LA) and a lower aorta/pulmonary artery ratio than ASD-only group patients. However, the right ventricular diameter (RVd) and right atrium were larger in patients with TAPVC than in those with only ASD. (III) The RVd was larger and the LA was smaller in patients with a PASP of ≥60 mmHg than in those with a PASP of <60 mmHg. (IV) Of those with a PASP of ≥60 mmHg, TAPVC patients had a smaller LA and a larger RVd than those with only ASD. (V) Pregnancy affected the PASP (adjusted odds ratio: 15.000, 95% confidence interval: 1.031–218.300, P=0.047). (VI) Echocardiography indicated that TAPVC patients with ASD had a right to left shunt at the atrial level and the pulmonary vein (PV) was not connected to the LA. Conclusions Searching for the PV by TTE is necessary for patients with ASDs, which may help avoid misdiagnosis. Moreover, pregnancy affects the PASP. Patients with TAPVC may present with a larger right heart, smaller LA, and lower aorta/pulmonary artery ration than those with only ASD.

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Section: Discussionmentioning

confidence: 99%

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults

Chen¹,

Gu²,

Han³

et al. 2023

J Thorac Dis

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“…Literature confirms the difficulties in complete assessment of PVs in TTEs. 7 Differential diagnosis of TAPVC includes ASD secundum, common atrium, PAPVC. In older patient mild cyanosis is present in case of TAPVC but this presentation is absent in case of ASD secundum and PAPVC unless there is associated pulmonary hypertension 8 .Depending on pulmonary venous obstruction there are two types of TAPVC.…”

Section: Discussionmentioning

confidence: 99%

Simple Radiography can Rescue from Dilemma

et al. 2019

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24 year old young lady came to us with a diagnosis of atrial septal defect (ASD) secundum who lost her follow up since her 4 years of age. Later her history, physical findings and investigations change the diagnosis into supracardiac total anomalous pulmonary venous drainage. Typical ‘snowman sign’ in chest x-ray and transthoracic echo helped to review the case. Proper history taking and careful evaluation by investigation is needful for each individual. Cardiovasc. j. 2019; 12(1): 75-78

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“…Total anomalous pulmonary venous drainage (TAPVD) is a rare congenital heart disease that accounts for approximately 1-3% of all congenital heart diseases [Hossein 2019;Yanqiang 2019;Yong 2017]. It can be divided into supra-cardiac, intra-cardiac, infra-cardiac, and mixed [Czekajska-Chehab 2018]. Few patients with TAPVD survive to adulthood.…”

Section: Introductionmentioning

confidence: 99%

Surgical Treatment of Total Anomalous Pulmonary Venous Drainage with Atrial Fibrillation in an Adult: A Case Report and Literature Review

Xiang

Wei

2022

HSF

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Total anomalous pulmonary venous drainage (TAPVD) is caused by the absence of the congenital connection between the pulmonary vein and left atrium. This causes blood drainage into the right atrium, resulting in poor development of the left atrium and increasing the burden for the right atrium. It is accompanied by an atrial septal defect. TAPVD mostly is diagnosed during the fetal period and rarely is reported in adults. Atrial fibrillation (AF), a common arrhythmia, originates primarily from the joint of the pulmonary vein and left atrium, whereas AF originating from the right atrium has not been documented. Herein, we report the case of a 45-year-old male diagnosed with TAPVD accompanied by AF. After the correction of TAPVD and radiofrequency ablation (RFA) for AF performed under general anesthesia and cardiopulmonary bypass, the patient returned to normal sinus rhythm and showed no AF recurrence during two years of follow up.

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Total anomalous pulmonary vein drainage in a 60-year-old woman diagnosed in an ECG-gated multidetector computed tomography – a case report and review of literature

Cited by 4 publications

References 22 publications

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults

Echocardiographic features and influencing factors of pulmonary hypertension of total anomalous pulmonary venous connection in adults

Simple Radiography can Rescue from Dilemma

Surgical Treatment of Total Anomalous Pulmonary Venous Drainage with Atrial Fibrillation in an Adult: A Case Report and Literature Review

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