Persistent lung shadow in an infant with ventricular septal defect and partial anomalous pulmonary venous connection associated with pulmonary venous obstruction

Background. Total anomalous pulmonary venous connection (TAPVC) is a critical congenital heart disease for which emergency surgery is required after birth. In cases of no intervention, TAPVC is associated with a high mortality rate in the first year of life. Although foetal echocardiographic techniques for diagnosing TAPVC have improved, TAPVC remains one of the most difficult congenital heart diseases to diagnose via foetal echocardiography. Here, we report a case of TAPVC with pulmonary venous obstruction (PVO), which was diagnosed via foetal echocardiography. Case Presentation. On foetal echocardiography at 32 weeks’ gestation, a large atrial septal defect, enlarged superior vena cava, and continuous flow pattern in the vertical vein from the common chamber were observed in the foetus. Paediatric cardiologists and cardiac surgeons, neonatologists, and obstetricians planned to perform a caesarean section and emergency heart surgery. The male infant was born at 37 weeks’ gestation via caesarean section, and postnatal echocardiography revealed PVO at the confluence of the superior vena cava and common chamber. Similarly, chest computed tomography confirmed the foetal diagnosis. The postnatal diagnoses were TAPVC type Ib, PVO, atrial septal defect, and patent ductus arteriosus. Surgical repair of the TAPVC was initiated within the first 3 hours of life. Screening brain echocardiography and head computed tomography revealed intracranial haemorrhage and hydrocephalus. Therefore, the patient underwent emergency bilateral external drainage on day 13. On day 48, a ventriculoperitoneal shunt was inserted owing to progressive brain ventricular dilatation. The patient was discharged home on postoperative day 68. Conclusions. Although the prognosis of TAPVC with PVO remains poor, continuous observation through foetal echocardiography and early interdepartmental collaboration can result in good outcomes.

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Total Anomalous Pulmonary Venous Connection with Severe Pulmonary Venous Obstruction

Circulation ◽

10.1161/01.cir.25.6.916 ◽

1962 ◽

Vol 25 (6) ◽

pp. 916-928 ◽

Cited By ~ 54

Author(s):

ALOIS R. HASTREITER ◽

MILTON H. PAUL ◽

MARIAN E. MOLTHAN ◽

ROBERT A. MILLER

Keyword(s):

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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Total anomalous pulmonary venous connection

ESC CardioMed ◽

10.1093/med/9780198784906.003.0196 ◽

2018 ◽

pp. 831-834

Author(s):

Marietta Charakida ◽

John Deanfield

Keyword(s):

Long Term Results ◽

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

Total anomalous pulmonary venous connection is repaired in childhood and the long-term results are usually excellent. Recent surgical advances have reduced further the rate of recurrent pulmonary venous obstruction.

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Surgical treatment of total anomalous pulmonary venous connection —Clinical aspects of pulmonary venous obstruction—

The Japanese Journal of Thoracic and Cardiovascular Surgery ◽

10.1007/bf03217887 ◽

1998 ◽

Vol 46 (11) ◽

pp. 1126-1132

Author(s):

Katsunori Yoshihara ◽

Tsukasa Ozawa ◽

Hiroshi Sakuragawa ◽

Takeshirou Fujii ◽

Noritsugu Shiono ◽

...

Keyword(s):

Surgical Treatment ◽

Pulmonary Venous Obstruction ◽

Clinical Aspects ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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The Warden Procedure through a Posterolateral Thoracotomy Approach

Texas Heart Institute Journal ◽

10.14503/thij-13-3474 ◽

2014 ◽

Vol 41 (5) ◽

pp. 499-501 ◽

Cited By ~ 1

Author(s):

Sowmya Ramanan ◽

Navaneetha Sasikumar ◽

Soman Rema Krishna Manohar ◽

Kotturathu Mammen Cherian

Keyword(s):

Superior Vena Cava ◽

Septal Defect ◽

Sinoatrial Node ◽

Superior Vena ◽

Vena Cava ◽

Posterolateral Thoracotomy ◽

Venous Obstruction ◽

Venae Cavae ◽

Anomalous Pulmonary Venous Connection ◽

Sinoatrial Node Dysfunction

The Warden procedure for the correction of a right-sided partial anomalous pulmonary venous connection to the high superior vena cava is well established. It has the advantages of avoiding sinoatrial node dysfunction and pulmonary and systemic venous obstruction. In the case related here, a 3-year-old girl presented with a superior vena cava type of sinus venosus atrial septal defect and an anomalously draining right upper pulmonary vein, with bilateral superior venae cavae. Our approach to the Warden procedure was through a right posterolateral thoracotomy, which provided additional advantages.

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Horseshoe lung associated with anomalous pulmonary venous connection without pulmonary hypoplasia

Cardiology in the Young ◽

10.1017/s1047951100011550 ◽

1995 ◽

Vol 5 (1) ◽

pp. 91-93 ◽

Cited By ~ 3

Author(s):

Antonio Corno ◽

Luca Rosti ◽

Ivan Machado

Keyword(s):

Ventricular Septal Defect ◽

Congenital Malformation ◽

Septal Defect ◽

Pulmonary Hypoplasia ◽

Caval Vein ◽

Superior Caval Vein ◽

Horseshoe Lung ◽

Anomalous Pulmonary Venous Connection

SummaryHorseshoe lung is an exceedingly rare congenital malformation, characterized by unilateral pulmonary hypoplasia together with a midline isthmus producing fusion of the tissues of the lower lobes. It is frequently associated with other cardiac and extracardiac anomalies. We report an infant with a variant of the horseshoe lung with partial anomalous venous connection of intracardiac type, ventricular septal defect, and persistent left superior caval vein. There was no pulmonary hypoplasia.

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In situ pericardium repair of pulmonary venous obstruction after repair of total anomalous pulmonary venous connection

The Japanese Journal of Thoracic and Cardiovascular Surgery ◽

10.1007/bf03032627 ◽

2002 ◽

Vol 50 (8) ◽

pp. 338-340 ◽

Cited By ~ 1

Author(s):

Hiroyuki Nishi ◽

Kyoichi Nishigaki ◽

Youichi Kume ◽

Katsuhiko Miyamoto

Keyword(s):

Pulmonary Venous Obstruction ◽

Venous Obstruction ◽

Anomalous Pulmonary Venous Connection

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MID -- TERM POST-OPERATIVE OUTCOME OF SINUS VENOSUS ATRIAL SEPTAL DEFECT WITH ANOMALOUS PULMONARY VENOUS CONNECTION WITH DIFFERENT SURGICAL STRATEGIES AND THEIR EFFECT ON SINUS NODE FUNCTION AND LATE VENOUS OBSTRUCTION.

10.22541/au.164156721.15230264/v1 ◽

2022 ◽

Author(s):

Vishal Bhende ◽

DR. AMIT KUMAR ◽

Badra Travedi ◽

DR. GURPREET PANESAR ◽

DR. B. VIRAL PATEL ◽

...

Keyword(s):

Atrial Septal Defect ◽

Septal Defect ◽

Sinus Node ◽

Sinus Venosus ◽

Venous Obstruction ◽

Surgical Strategies ◽

Operative Outcome ◽

Anomalous Pulmonary Venous Connection

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Neonatal repair of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome: a case report

Journal of Cardiothoracic Surgery ◽

10.1186/s13019-021-01722-5 ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Takahiro Ito ◽

Ikuo Hagino ◽

Mitsuru Aoki ◽

Kentaro Umezu ◽

Tomohiro Saito ◽

...

Keyword(s):

Computed Tomography ◽

Pulmonary Veins ◽

Median Sternotomy ◽

Bronchial Tree ◽

Pulmonary Venous Obstruction ◽

Goldenhar Syndrome ◽

Venous Obstruction ◽

Vertical Vein ◽

Lung Agenesis ◽

Anomalous Pulmonary Venous Connection

Abstract Background Total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome is extremely rare. Case presentation We present a case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome in a patient who was diagnosed based on transthoracic echocardiography and computed tomography. We observed complete absence of the lung, the bronchial tree, and vascular structures on the right side, with abnormal drainage of the left pulmonary veins into the innominate vein. The patient showed clear clinical evidence of pulmonary venous obstruction and underwent surgery 3 days after birth. The pulmonary venous chamber containing the vertical vein was anastomosed to the left atrium using 7–0 PDS running sutures via a median sternotomy. Echocardiography and computed tomography performed 1 year postoperatively revealed no pulmonary venous obstruction. Conclusion We report a rare case of total anomalous pulmonary venous connection accompanied by unilateral lung agenesis and Goldenhar syndrome, which was successfully repaired 3 days after birth. A median sternotomy is a safe and effective approach for surgical repair of congenital heart disease with unilateral lung agenesis. Repair of the supra cardiac total anomalous pulmonary connection using the vertical vein is feasible in patients with a small pulmonary venous chamber.

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