Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update

Kao, Chuan-Chi; Hsieh, Chia Chien; Cheng, Po‐Jen; Cw, Chiang; Huang, Shu‐Mei

doi:10.1016/j.jmu.2017.08.002

Cited by 37 publications

(50 citation statements)

References 36 publications

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“…TAPVC is one of the few diseases that require emergency treatment among CHDs . Operation in hemodynamically unstable neonates is expected to yield high mortality and morbidity rates.…”

Section: Discussionmentioning

confidence: 99%

“…Total anomalous pulmonary venous connection (TAPVC) is congenital heart disease (CHD), accounting for approximately 1% to 1.5% of all CHDs. [1][2][3][4] It is sometimes associated with a univentricular heart or heterotaxy syndrome, both of which are consequently associated with high morbidity and mortality rates. 1,[3][4][5][6][7][8][9][10] In contrast, isolated TAPVC is known to have a good prognosis.…”

Section: Introductionmentioning

confidence: 99%

“…[1][2][3][4] It is sometimes associated with a univentricular heart or heterotaxy syndrome, both of which are consequently associated with high morbidity and mortality rates. 1,[3][4][5][6][7][8][9][10] In contrast, isolated TAPVC is known to have a good prognosis. 3,5,6,[9][10][11] Nevertheless, isolated TAPVC may be associated with higher mortality and morbidity rates than other CHDs because obstructive flows often require emergency operation within days after birth.…”

Section: Introductionmentioning

confidence: 99%

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Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single‐secondary center experience

et al. 2019

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Background and Aim of the Study: Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart defect. This study aimed to evaluate the outcome of isolated TAPVC repairs and the prognoses of affected patients in the last 12 years at a single center. Methods: We retrospectively analyzed the medical records of 51 patients who underwent isolated TAPVC repair from 2007 to 2018. Results: The median age at operation was 19 days, and the median body weight was 3.3 kg. Thirteen (25.5%) patients had emergency operations, and the median followup period was 29.54 ± 36.77 months. Early mortality was noted in five patients and late mortality was noted in one patient. Pulmonary vein stenosis was observed in 22 patients within 3 to 6 months after the operation, and six patients required reoperation or transcatheter interventions. Low birth weight, small left atrial volume, long operation time, and preoperative heart failure were identified as risk factors for mortality.Conclusions: Isolated TAPVC can rapidly lead to hemodynamic instability during the neonatal period and is associated with high mortality rates. Increasing the prenatal diagnosis rate and stabilizing the patients' condition before the operation are considered important for improving the surgical outcome. K E Y W O R D S preoperative management, pulmonary vein stenosis, surgical outcome, total anomalous pulmonary venous connection

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“…TAPVC is one of the few diseases that require emergency treatment among CHDs . Operation in hemodynamically unstable neonates is expected to yield high mortality and morbidity rates.…”

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single‐secondary center experience

et al. 2019

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“…It is characterized by the absence of connection between the pulmonary veins and the morphologic left atrium (LA). Although it is a rare CHD (incidence rate of <1% of all CHDs), prenatal diagnosis of TAPVC is crucial for adequately planning delivery and postnatal management, especially in critical forms such as obstructive TAPVC 11,12 . However, according to previous studies, diagnosing anomalous pulmonary venous connection (APVC) in utero is challenging, with 1.9% to 10% rate, especially when it is not associated with other defects 13,14 .…”

Section: Introductionmentioning

confidence: 99%

Prenatal diagnosis of total anomalous pulmonary venous connection: 2D and 3D echocardiographic findings

Bravo-Valenzuela

Peixoto

Júnior

2021

J of Clinical Ultrasound

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Total anomalous pulmonary venous connection (TAPVC) is a rare cyanotic congenital heart disease that accounts for less than 1% of all congenital heart diseases. TAPVC is characterized by the absence of pulmonary venous drainage into the left atrium. It is underdiagnosed in utero, with prenatal detection rates of less than 1.4%. The prenatal diagnosis of TAPVC affects the postnatal outcome, particularly in obstructive forms (critical TAPVC), since planned delivery and perinatal management are mandatory. Thus, identifying the ultrasound key markers of TAPVC is important for the prenatal diagnosis and perinatal assistance. The ventricular size discrepancy (right ventricle > left ventricle) can be a useful marker. Furthermore, the increased retroatrial distance between the left atrium and the aorta could be a diagnostic marker for prenatal TAPVC, especially when the left atrium is small. Three‐ (3D) and four‐dimensional ultrasonography may improve the prenatal diagnosis of TAPVC. This study reviews the two (2D) and 3D ultrasonographic markers used in the antenatal diagnosis of TAPVC, with a focus on the tools that can be used by sonographers, obstetricians, and fetal medicine specialists to improve the prenatal diagnosis of TAPVC, and when to refer the case to a fetal cardiac specialist.

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“…The infra-cardiac variation is associated with the confluence of all pulmonary veins, which, through a descending vertical vein (VV) passing behind the LA, drain sub-diaphragmatically into splanchnic veins. This defect commonly presents in neonates with right heart failure and pulmonary venous obstruction [ 1 ]. There is variation in the surgical approach to the VV during repair of TAPVC; a number of published cases report persistent enlargement of the VV when flow through a VV is maintained at the time of repair.…”

Section: Introductionmentioning

confidence: 99%

Unligated vertical vein presenting as a large atrio-portal shunt in adulthood: a case report

Layton

Koulouroudias

Issa

et al. 2020

Journal of Surgical Case Reports

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A 28-year-old male with infra-cardiac totally anomalous pulmonary venous connection (TAPVC) repaired as new-born presented in adulthood with right heart strain and very large left atrium to portal vein vessel. Residual connections from pulmonary veins to systemic circulation are believed to represent persistent ‘vertical veins’ (VV) not ligated at the time of the initial surgery. In our patient, since endovascular occlusion was not judged suitable, the anomalous vessel was surgically ligated and resected. A review of the literature failed to find such a procedure reported in an adult patient and analyzed the intra-operative ligation of VV during repair of TAPVC.

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Total Anomalous Pulmonary Venous Connection: From Embryology to a Prenatal Ultrasound Diagnostic Update

Cited by 37 publications

References 36 publications

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single‐secondary center experience

Assessing the recently noted surgical outcome of isolated total anomalous pulmonary venous connection repair: A single‐secondary center experience

Prenatal diagnosis of total anomalous pulmonary venous connection: 2D and 3D echocardiographic findings

Unligated vertical vein presenting as a large atrio-portal shunt in adulthood: a case report

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