Robotic-assisted two-patch repair of right partial anomalous pulmonary venous connection and sinus venosus defect

Abstract Introduction: The single- and double-patch repairs are undoubtedly 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 paediatric and adult patients, focusing in particular on the occurrence of ectopic atrial rhythm. Material and methods: Seventy patients (male: 38, 54.2%) underwent surgical repair for partial anomalous pulmonary venous connection with sinus venosus atrial septal defect. Forty-nine patients (70%) underwent surgical repair in paediatric age (<16 years old), while 21 of (30%) patients were operated in adulthood. Thirty patients (42.8%) underwent single-patch repair and 39 patients (55.7%) underwent double-patch repair. In only one patient, the Warden procedure was performed (1.4%). Median follow-up time was 52 months (IQ 15.1–113). Results: The type of surgical technique didn’t affect the incidence of ectopic atrial rhythm (26.6% in single-patch group and 25.6% in double-patch groups, p = 0.9). At long-term follow-up, ectopic atrial rhythm, as an expression of sinoatrial node disturbance, was however significantly more frequent in the paediatric population (28.8% paediatric group and 4.7% adult group, p = 0.02). Conclusions: The higher incidence of ectopic atrial rhythm in children is probably related to the closer position of the sinus node to the superior cavoatrial incision, which makes irreversible iatrogenic traumatism more likely to occur. Surgical techniques that avoid any manipulation on the superior cavoatrial junction should, therefore, be preferred for children undergoing partial anomalous pulmonary venous connection repair.

Download Full-text

Sinus Venosus Defect and Partial Anomalous Pulmonary Venous Connection: Effect of Surgical Strategies on Sinus Node Function and Potential Obstruction of the Superior Vena Cava

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-0035-1544525 ◽

2015 ◽

Vol 63 (S 01) ◽

Author(s):

P. Filzmaier ◽

I. Knez ◽

D.-E. Malliga ◽

H. Mächler ◽

M. Wallner ◽

...

Keyword(s):

Superior Vena Cava ◽

Superior Vena ◽

Sinus Node ◽

Vena Cava ◽

Sinus Venosus ◽

Surgical Strategies ◽

Sinus Venosus Defect ◽

Anomalous Pulmonary Venous Connection

Download Full-text

Preliminary analysis of arrhythmias in sinus venosus defect with Partial Anomalous Pulmonary Venous Connection (PAPVC)

The Thoracic and Cardiovascular Surgeon ◽

10.1055/s-2005-862091 ◽

2005 ◽

Vol 53 (S 01) ◽

Author(s):

S Buz ◽

V Alexi-Mekhishvili ◽

F Dorbar ◽

P Lange ◽

R Hetzer

Keyword(s):

Preliminary Analysis ◽

Sinus Venosus ◽

Sinus Venosus Defect ◽

Anomalous Pulmonary Venous Connection

Download Full-text

Long-Term Outcomes of the Double-Barrel Technique for Superior Sinus Venosus Defect With Partially Anomalous Pulmonary Venous Connection

World Journal for Pediatric and Congenital Heart Surgery ◽

10.1177/2150135120938352 ◽

2020 ◽

Vol 11 (6) ◽

pp. 733-741

Author(s):

Ujjwal Kumar Chowdhury ◽

Srikant Sharma ◽

Lakshmi Kumari Sankhyan ◽

Niwin George ◽

Sukhjeet Singh ◽

...

Keyword(s):

Superior Vena ◽

Pulmonary Veins ◽

Vena Cava ◽

Growth Potential ◽

Sinus Venosus ◽

Long Term Outcomes ◽

Sinus Venosus Defect ◽

Anomalous Pulmonary Venous Connection

Background: Repair of superior sinus venosus defect with high partially anomalous pulmonary venous connection (PAPVC) using an intracardiac baffle may be complicated by systemic or pulmonary venous pathway obstruction and sinus nodal dysfunction (SND). Our surgical strategy for repair of all types of superior sinus venosus defect has evolved chiefly to avoid the abovementioned complications and preserving the growth potential of the superior cavoatrial junction. Methods: Between 2007 and 2019, fifty consecutive patients aged 2 to 60 (mean, 17.6±16.7) years underwent repair of superior sinus venosus defect using the double-barrel technique as described. The anomalous pulmonary veins drained into the superior cavoatrial junction in 17 patients and more than 2 cm above the cavoatrial junction in 33 patients. Results: There were no early or late deaths and no reoperations. At a mean follow-up of 103.9 (±50.2) months, all survived the operation, and actuarial freedom from SND was 97.9% (±standard error, 0.02%; 95% CI: 0.86-0.99). No patient had systemic or pulmonary venous pathway obstruction. A permanent pacemaker was required in one (2%) patient for sick sinus syndrome. Conclusions: The double-barrel method is an expedient, safe, and effective technique in superior sinus venosus defect. It provides dual drainage of superior vena cava preserving the superior cavoatrial junction without causing systemic or pulmonary venous pathway obstruction and can be utilized in all cases including those with high PAPVC. Preservation of the cavoatrial junction and use of autogenous atrial tissue for systemic venous pathway avoids SND and preserves growth potential.

Download Full-text

Abstract 14310: Elephant Sitting on My Chest: A Tale of Two Vessels

Circulation ◽

10.1161/circ.142.suppl_3.14310 ◽

2020 ◽

Vol 142 (Suppl_3) ◽

Author(s):

Anudeep K Dodeja ◽

Patrick I McConnell ◽

Elisa A Bradley ◽

Curt Daniels ◽

Laxmi S Mehta ◽

...

Keyword(s):

Pulmonary Artery ◽

Symptomatic Relief ◽

Pulmonary Veins ◽

Main Pulmonary Artery ◽

Laboratory Data ◽

Venous Drainage ◽

Sinus Venosus ◽

Exercise Intolerance ◽

Extrinsic Compression ◽

Sinus Venosus Defect

Introduction: A dilated main pulmonary artery (MPA) seen in congenital heart disease (CHD) or pulmonary arterial hypertension (PAH) is a rare cause of angina due to left main coronary artery (LMCA) compression. Case: A 49 year old male with no prior cardiac history presented with substernal chest pain. He reported exercise intolerance for several years. On exam he had an oxygen saturation of 85%, loud P2, fixed split second heart sound and ejection click. Electrocardiogram showed right ventricular (RV) hypertrophy and no significant ST changes. Laboratory data revealed a negative troponin. An enlarged main and right pulmonary artery (RPA) (Figure 1A) was seen on computed tomography (CT) chest angiography for pulmonary embolism (CTPE) and on echocardiography he had a severely dilated RV with decreased function (Figure 1B,1C). Cardiac catheterization showed a step up in saturations from 56 to 71%, Qp:Qs of 1.5 mean MPA pressure of 53 mmHg, pulmonary capillary wedge pressure of 11mmHg and pulmonary vascular resistance (PVR) of 7WU. A slit-like narrowing of LMCA ostium was seen on coronary angiography and intravascular ultrasound (Figure 1D). He was diagnosed with a sinus venosus defect, anomalous right upper pulmonary venous drainage and suspicion for extrinsic compression of LMCA by the dilated RPA that was confirmed on cardiac CT (Figure 1E-I). He was started on triple therapy for PAH with a significant reduction in PA pressures (mean 36 mmHg and PVR 2.1 WU). Percutaneous stenting of the LMCA was deferred and he underwent successful sinus venosus defect repair with a 5mm fenestrated patch, baffling of the anomalous pulmonary veins and a 32 mm interposition graft in the MPA with reduction plasty of MPA/RPA. Conclusion: This is an unusual presentation of unrepaired CHD with angina due to a compressed LMCA from a dilated pulmonary artery. Treatment of the underlying PAH as well as repair of the defect including reduction plasty of the MPA is a consideration for symptomatic relief.

Download Full-text