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Ganapathy Krishnan Subramaniam1, Pradeep Gnanasekaran1, Dhruva Sharma2, Ramya Shri3, Anitha Chandrashekhar1
1 Institute of Heart and Lung Transplant and Mechanical Circulatory Support, MGM Healthcare, Chennai, Tamil Nadu, India
2 Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, Jaipur, Rajasthan, India
3 Department of Paediatric Cardiology, MGM Healthcare, Chennai, Tamil Nadu, India
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Date of Submission27-Jul-2021Date of Decision20-Sep-2021Date of Acceptance12-Jun-2022Date of Web Publication06-Jan-2023
Abstract
The presence of systemic and pulmonary venous anomalies in late presenting transposition of great arteries (TGA) may make standard atrial switch procedures difficult, necessitating modification in the surgical procedure. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein with TGA and regressed left ventricle, where a combination of Warden procedure and Mustard procedure was used.
Keywords: Combined warden and mustard procedure, partial anomalous pulmonary venous connection, transposition of great arteries
How to cite this article: Introduction 
Case of late presenting transposition of great arteries (TGA) is usually managed with a palliative atrial switch. When there is an associated anomalous pulmonary vein draining into the superior vena cava (SVC), the atrial switch gets complicated to create an obstruction-free pathway. In this background, modified Mustard's procedure including the right atrial appendage within systemic venous baffle combined with the warden's procedure can alleviate the technical challenge. We present a case report of a 13-year-old boy with a partial anomalous connection of right superior pulmonary vein (RSPV) with TGA and regressed left ventricle (LV), where a combination of Warden procedure and Mustard procedure was used.
Case Report A 30 kg, 13-year-old boy, with TGA and regressed LV, presented for the first time with a saturation of 50%–60%, hemoglobin of 22 g/dl, and severe functional incapacitation. Computerized tomography showed that there was anomalous pulmonary venous drainage of the RSPV into the SVC-partial anomalous pulmonary venous connection (PAPVC) and there was a small secundum atrial septal defect (ASD) [Figure 1]a and [Figure 1]b. There was no sinus venosus defect. In addition, he had thrombocytopenia and deranged prothrombin time attributable to his long-standing cyanosis.
Figure 1: (a and b) Preoperative computed tomography scan showing the partial anomalous connection of right upper pulmonary vein with superior vena cavaIntraoperatively, the saturation in the pulmonary artery was 90% with systemic saturation of 60%. It was decided to proceed with a modified Mustard procedure-incorporating the right atrial appendage in the systemic venous baffle and performing a Warden procedure connecting the SVC which was divided above RSPV to the right atrial appendage [Figure 2].
Figure 2: Intra-operative photograph showing the baffle created within the right atrium using PTFE and the Warden anastomosis for PAPVC repair, PTFE: Polytetrafluoroethylene, PAPVC: Partial anomalous pulmonary venous connectionThe patient was put on cardiopulmonary bypass with innominate vein and low inferior vena cava (IVC) and aortic cannulation and heart arrested with del Nido cardioplegia. The right atrium was opened and septum excised, and a 20 mm polytetrafluoroethylene (PTFE) tube was cut open to fashion the systemic venous baffle, with one end sutured to the left atrium anterior to left pulmonary venous opening and the other end sutured to the right atrial appendage and the anterior part of the interatrial septum. The IVC is baffled to the mitral valve by suturing the opened up tube anterior to the pulmonary veins across the floor of the left atrium, around the mouth of IVC, climbing on the anterior aspect of the atrial septum. The opening of the coronary sinus opens up this pathway. The SVC was divided above the opening of the pulmonary veins and was anastomosed to the right atrial appendage, with an anterior autologous pericardial patch to reduce the tension on the anastomosis. The right atrial incision was then closed after further excising the posterior part of the interatrial septum, ensuring unobstructed pulmonary venous drainage to the tricuspid valve. The patient had an uneventful intraoperative and postoperative course and was discharged on the 7th day.
Discussion Atrial switch is an important tool for managing isolated atrioventricular discordance, as part of double switch procedure for corrected TGA, for managing delayed presenting TGA with an intact septum or in situations where arterial switch procedure is prohibitively risky and in palliating Eisenmenger patients with transposition physiology. The advantage with “Swiss roll Senning's” procedure using in situ pericardium, is the growth potential of the tissues with reduced chance of baffle problems and atrial arrhythmia. However, in the juxtaposition of atrial appendages and pulmonary and systemic venous anomalies, especially in older children, “Mustard procedure” has a role to play.
The association of PAPVC with TGA is a rare entity. Left-sided PAPVC in association with late presenting TGA has been reported before which was managed by modifying Senning procedure after anastomosing the left vertical vein to the left atrial appendage.[1] Another neonatal case with anomalous RSPV connection with sinus venous ASD was managed by two patch technique repair and arterial switch.[2]
In our case, right PAPVC without sinus venous ASD and small secundum ASD provided some palliation resulting in late presentation. The modified Mustard procedure, incorporating the right atrial appendage in the Mustard baffle was recently substantiated to produce better flow dynamics than classic Mustard by the Yacoub group.[3] Warden procedure is to repair PAPVC, connecting the SVC to the atrial appendage. The current procedure incorporates the features of both these techniques [Figure 3].
Figure 3: Showing sketch diagram of the direction of systemic and pulmonary venous blood flow after the combined Warden and MustardDr Bacha E has hailed that the modified Mustard procedure, could be an important contribution to pediatric cardiac surgery in the middle- and low-income countries and would be an important surgery in the armamentarium of surgeons dealing with late presenting TGA.[4]
The important component of Yacoub's modification of the Mustard procedure is to limit the atrial incision to the posterior and inferior aspect of the right atrium anterior to crista, maximizing the atrial muscle in the systemic venous baffle. The reservoir and contractile function of the atrial tissue has been shown to improve the flow patterns using computational fluid dynamics.[3] Before the warden procedure, right atrial appendage trabeculation needs to be divided to ensure the smooth flow of SVC blood into the atrial appendage. An anterior patch was used to reduce the tension-free anastomosis.[5] The excision of the posterior part of the atrial septum has to be complete. We used an opened-up PTFE tube to fashion the pathway. As the child was “grown up” and the tube would provide shape and avoid collapse and kink of the systemic venous baffle.
If uncorrected, the extrapulmonary venous blood in the systemic venous baffle could have resulted in volume loading of the already regressed LV which was used to receive predominantly the left pulmonary venous blood.
Conclusion In summary, the current case report combines Warden and Modified Mustard procedure, to manage late presenting transposition with PAPVC.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's parents have given his consent for his images and other clinical information to be reported in the journal. The patient's parents understand that his names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
References 
Correspondence Address:
Dr. Dhruva Sharma
Department of Cardiothoracic and Vascular Surgery, SMS Medical College and Attached Hospitals, J L N
Marg, Jaipur - 302 001, Rajasthan
India
Source of Support: None, Conflict of Interest: None
CheckDOI: 10.4103/apc.apc_152_21
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