Total Anomalous Pulmonary Venous Connection

Shi, Guoyong; Zhu, Zhongqun; Chen, Jimei; Ou, Yanqiu; Hong, Haifa; Nie, Zhiqiang; Zhang, Haibo; Liu, Xiaoqing; Zheng, Jie; Sun, Qiang; Liu, Jinfen; Chen, Huiwen; Zhuang, Jian

doi:10.1161/circulationaha.116.023889

Cited by 206 publications

(77 citation statements)

References 24 publications

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“…In normal fetuses, the space posterior to the left atrium (LA) is usually narrow, with the descending aorta and being close to each other . We previously reported that this space is wider in fetuses with total anomalous pulmonary venous connection (TAPVC), a rare form of severe congenital heart disease that requires emergency neonatal surgery associated with a high mortality . It is still difficult to detect TAPVC prenatally because the four‐chamber view looks normal .…”

Section: Introductionmentioning

confidence: 99%

Evaluation of the post‐LA space index in the normal fetus

et al. 2019

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Objective We previously reported the post‐LA space index (PLAS index), which is calculated as left atrial‐descending aorta distance (LD) divided by the diameter of descending aorta (DA), could be useful for prenatal diagnosis of total anomalous pulmonary venous connection (TAPVC). In this study, we evaluated PLAS index in normal fetuses to assess its usefulness. Methods In 304 normal fetuses, LD and DA were retrospectively measured, and the PLAS index was calculated. In 206 fetuses with data on the biparietal diameter (BPD) and femoral length (FL), the relationship between the PLAS index and them was investigated. We also calculated the PLAS index in 13 TAPVC fetuses. Results Mean LD was 3.0+/−0.94 mm, mean DA was 4.8+/−0.87 mm, and the mean PLAS index was 0.62+/−0.19. DA and LD were correlated with gestational age (R = 0.52, 0.25), while the PLAS index showed little variation with gestational age (R < 0.2). BPD and FL were correlated with DA (R = 0.4, 0.42) but not with LD or the PLAS index (both R < 0.2). In the TAPVC fetuses, both LD and the PLAS index were significantly higher than in normal fetuses. Conclusion In normal fetuses, the PLAS index was independent of gestational age and fetal physique and may be useful for raising the suspicion of congenital heart disease, including TAPVC.

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Section: Introductionmentioning

confidence: 99%

Evaluation of the post‐LA space index in the normal fetus

et al. 2019

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“…Other rare cases involving TAPVC were also reported in the specialty literature, such as the association between tetralogy of Fallot with anomalous left coronary artery from pulmonary artery and totally anomalous pulmonary venous connection, [ 22 ] or the coexistence of TAPVC and persistent left superior vena cava. [ 23 ] According to Shi et al [ 24 ] obstructed TAPVC was discovered in 25% of newborns, the mean surgical age were about 215 days of life with 51 deaths of the 768 cases with different types of anomalous pulmonary venous connection.…”

Section: Case Reportmentioning

confidence: 99%

Prenatal diagnoses of an uncommon isolated obstructed supracardiac total anomalous pulmonary venous connection

Muntean

Mărginean

Stanca³

et al. 2017

Medicine

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Introduction:Total anomalous pulmonary venous connection is an uncommon congenital heart disease. Four types are described based on the site of pulmonary venous drainage: supracardiac, cardiac, infradiaphragmathic, and mixed connection. In most cases of supracardiac type, the common venous confluence drains through an ascending left vertical vein into the brachiocephalic vein, right superior vena cava, and then into the right atrium. Anomalous connection of the pulmonary venous confluence directly to the right SVC, especially the obstructed form is an unusual and severe supracardiac variant. The prenatal diagnosis is challenging.Patient concerns:We present a case report of a fetus diagnosed with TAPVC at 23 gestational weeks.Diagnosis interventions:The 4-chamber view showed a small left atrium, with a “smooth” posterior wall and the absence of pulmonary vein connection. This is the first case of prenatally diagnosed isolated, obstructed supracardiac type with drainage directly into the right superior vena cava.Conclusion:The obstetrician and fetal cardiologist should be cautious at the direct and indirect echocardiographic signs of this condition. A prenatal diagnose of isolated, obstructed form is important for adequate planning of delivery and postnatal surgery in a tertiary center.

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“…However, the therapeutic effect of this operation is poor in physiology and hemodynamics. In addition, pulmonary vein obstruction (PVO) is one of the most frequently stated problems with postoperative death (Seale et al, 2013;Hoashi et al, 2015;Shi et al, 2017).…”

Section: Introductionmentioning

confidence: 99%

Numerical Simulation of Hemodynamics in Two Models for Total Anomalous Pulmonary Venous Connection Surgery

et al. 2020

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Objective: To numerically compare the prospective hemodynamic outcomes between a new window surgery and a traditional surgery in the treatment of supracardiac total anomalous pulmonary venous connection (S-TAPVC). Methods: A 3D geometry model, composed of pulmonary vein (PV) and left atrium (LA), was reconstructed based on summarized data with S-TAPVC. Two surgery models were established based on this model. One is the traditional surgery model, where an elliptical anastomosis was created by incising and stitching the LA and the common vein (CV) along the axis of the CV. The other is the new window surgery model, where the CV was incised with an H-shaped orifice, and LA was incised with a transposed H-shaped orifice, and then the orifice edges were stitched like a window. Two models with a relative cross sectional area (RCSA) of 300 mm 2 /m 2 and 500 mm 2 /m 2 were established, which correspond to traditional surgery and window surgery. Numerical simulation of hemodynamics was carried out. The velocity, left atrium and pulmonary vein pressure, the pressure difference of anastomosis and the energy conversion efficiency were analyzed to evaluate the prospective hemodynamic outcomes of these two operations. Results: Window surgery presented a lower blood flow velocity, pressure difference, and the WSS at the anastomosis, compared to traditional surgery. In terms of energy loss, the power conversion efficiency of window surgery was significantly higher than that of traditional surgery, with 66.8% and 53.5%, respectively. Conclusion: The new window surgery demonstrates a lower pressure difference of anastomosis and higher energy conversion efficiency, which may be a better choice compared with the traditional surgery for S-TAPVC patient.

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Total Anomalous Pulmonary Venous Connection

Abstract: Surgical correction in patients with TAPVC with a biventricular anatomy can achieve an acceptable outcome. Risk factors such as a younger age at the time of repair, infracardiac and mixed TAPVC, and preoperative PVO were associated with a poorer prognosis.

Cited by 206 publications

References 24 publications

Evaluation of the post‐LA space index in the normal fetus

Evaluation of the post‐LA space index in the normal fetus

Prenatal diagnoses of an uncommon isolated obstructed supracardiac total anomalous pulmonary venous connection

Numerical Simulation of Hemodynamics in Two Models for Total Anomalous Pulmonary Venous Connection Surgery

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