Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Szychta, Wojciech; Cerin, F.E.S.C. Gheorghe; Popa, Bogdan Adrian; Armienti, Felice; Lanzillo, Guido; Diena, Marco; Opolski, Grzegorz

doi:10.1111/echo.12863

Cited by 7 publications

(8 citation statements)

References 7 publications

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“…We present a rare case of atypical anatomical variation of PAPVC, which was overlooked in the beginning. The initial diagnosis of most PAPVC is often made by TTE [Szychta 2015]. However, this case was delayed due to misdiagnosis by TTE both at our hospital and at a local hospital.…”

Section: Discussionmentioning

confidence: 89%

Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension

Wei

Zheng

et al. 2017

HSF

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In this case report, we present the delayed diagnosis of an atypical partial anomalous pulmonary venous connection, which was initially misdiagnosed as primary pulmonary arterial hypertension. It was difficult to make a conclusive diagnosis using transthoracic echocardiography (TTE), because the two high right superior pulmonary veins drained into the superior vena cava and limited the shunt of patent foramen ovale. Preoperative TTE only showed right heart volume overload, pulmonary arterial hypertension, and severe tricuspid valve insufficiency. A chest CT-angiography (angio-CT) finally found the anomalous right superior pulmonary venous connection, which was further confirmed by surgery. The postoperative course was uneventful.

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Section: Discussionmentioning

confidence: 89%

Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension

Wei

Zheng

et al. 2017

HSF

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“…In 90% of cases, right-sided PAPVC is associated with a sinus venosus type atrial septal defect (ASD), in most instances located near the superior cavo-atrial junction (5). An intact interatrial septum is observed in only 3% of patients with PAPVC.…”

Section: Introductionmentioning

confidence: 99%

The impact of the superior cavo-atrial incision in children and adults.

Ait-Alì¹,

Ravaglioli²,

Festa³

et al. 2020

Preprint

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Background and aim of the study The single-and double-patch repairs are the most commonly used techniques for the surgical management of partial anomalous pulmonary venous connection associated with sinus venosus atrial septal defect. The aim of this study was to retrospectively compare early and long-term surgical outcomes in pediatric and adult patients, focusing in particular on the occurrence of postoperative rhythm disturbances. Methods 115 patients (male: 63, 54.8%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. 80 patients (70.4%) underwent surgical repair in pediatric age (<16 years old), while 35 (29.6%) patients were operated in adulthood. 49 patients (42.6%) underwent single-patch repair and 65 patients (56.5%) underwent double-patch repair. In only one patient the Warden procedure was performed (0,9%). Median follow-up time was 52 months (IQ 15.1-113). Results The type of surgical technique didn't affect the overall incidence of all rhythm disturbances (41% in single patch group and 35% in double patch groups, p=0.5). At long-term follow-up, ectopic atrial rhythm, as expression of sinoatrial node disturbance, was however significantly more frequent in the pediatric population (32% pediatric group and 8% adult group, p=0.02). Conclusions The higher incidence of ectopic atrial rhythm in children is probably related to the closer relationship of the sinus node to the superior cavo-atrial incision, which makes iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavo-atrial junction should therefore be preferred for children undergoing partial anomalous pulmonary venous connection repair. The impact of the superior cavo-atrial incision in children and adults.

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“…1 Clinically, 1 out of 10 atrial septal defects is associated with partial anomalous pulmonary venous connection (PAPVC), 2,3 and approximately 90% of sinus venosus defects are accompanied by PAPVC. 4,5 PAPVC itself occurs with a prevalence of approximately 3% of all CHD. 6 It is defined as a drainage of one or more pulmonary veins to the right atrium or to one of its tributaries.…”

Section: Introductionmentioning

confidence: 99%

“…20 Pulmonary hypertension is a possible consequence of a left-to-right-shunt. 5,8,21 Furthermore, a leftto-right-shunt can lead to ventricular failure and death. 8,19,21 The purpose of this analysis is to compare the outcome of the four surgical therapies for PAPVC, single-patch, doublepatch, Warden technique and direct-anastomosis, regarding their complications, such as pulmonary hypertension, right ventricular dysfunction, heart rhythm disorders and venous stenosis.…”

Section: Introductionmentioning

confidence: 99%

A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience

Jaschinski

Cussigh

Fonseca

et al. 2019

Thorac cardiovasc Surg

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Background Different methods for surgical correction of partial anomalous pulmonary venous connection (PAPVC) exist. We evaluated the outcomes of four techniques regarding morbidity and mortality. Methods A total of 116 patients underwent PAPVC repair in our institution over a period of 20 years. Single-patch technique (n = 82 [71%], mean age: 18.59 ± 20.49 years), double-patch technique (n = 13 [11%], mean age: 43.18 ± 25.14 years), Warden's technique (n = 7 [6%], mean age: 10.04 ± 10.47 years), and direct implantation of anomalous pulmonary veins (n = 14 [12%], mean age: 14.42 ± 18.58 years) were examined. Results Out of the 116 patients, one patient (0.9%) developed pulmonary hypertension after discharge and three patients (2.6%) with normal right cardiac function showed right ventricular failure. In total, a pacemaker was inserted in seven cases (6%). Three patients (2.6%) presented with persistent nonsinus rhythm during follow-up. This complication was most frequently seen in the double-patch group being significantly increased compared with the other groups (p = 0.035). One patient presented with a mild stenosis of the superior vena cava. There were two early, nonsurgery-related deaths and no late mortality. Conclusion Operative therapy of PAPVC has low postoperative morbidity and mortality. Therefore, surgical repair of this cardiac anomaly is a safe and reproducible treatment independent of the applied method. The surgical technique must be selected based on the anatomy and possible accompanying congenital heart defects. Special care should be taken when using the double-patch technique because of significant more frequent nonsinus rhythm events postoperatively.

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Atypical Abnormal Pulmonary Vein Drainage with Atrial Septal Defect: Surgical Treatment

Cited by 7 publications

References 7 publications

Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension

Atypical Case of Partial Anomalous Pulmonary Venous Connection Misdiagnosed as Primary Pulmonary Arterial Hypertension

The impact of the superior cavo-atrial incision in children and adults.

A Wide Comparison of Techniques for Repair of PAPVCs: One Institution's 20-Year Experience

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