Cox-Maze IV Procedure Concomitant with Valvular Surgery In Situs Inversus Dextrocardia: A Single-Center Experience in China
Summary
We summarize the Cox-Maze IV procedure concomitant with valvular surgery performed in patients with situs inversus dextrocardia at this institution.
Abstract
Atrial fibrillation (AF) is the most common cardiac arrhythmia. The use of ablation technologies made the Cox-Maze IV procedure (CMP-IV) technically easier, faster, becoming the gold standard for the surgical treatment of AF. However, the efficacy and safety of CMP-IV in situs inversus dextrocardia are largely unknown. This paper summarizes the CMP-IV procedure performed concomitantly with valvular surgery in patients with situs inversus dextrocardia at this institution.
From February 2016 to September 2020, three dextrocardia patients with persistent AF and valvular diseases were referred to this institution for valvular and CMP-IV surgery. CMP-IV was performed using either cryoablation with a nitrous oxide (N2O)-based cryoprobe or a bipolar radiofrequency clamp and bipolar radiofrequency pen. Mechanical valve replacement or mitral vavuloplasty was performed in another patient in addition to tricuspid annuloplasty. Transmurality of the ablated atrial tissues was evaluated by electron microscopy. Heart function was assessed by transthoracic echocardiography. Cardiac rhythm was monitored by 24 h Holter at 3, 6, 12, 18, 24, and 48 months follow-up.
All the AF was successfully eliminated in the ablation procedure without recurrence or other complications during hospitalization. The mean bypass and crossclamp times were similar in all the patients. The postoperative ventilator support time, the duration of stay in the ICU, and postoperative residence time were also not significantly different among the patients. Transmural atrial necrosis was detected in the ablated atrial tissues. Sinus rhythm maintenance was achieved at 3, 6, 12, 18, 24, and 48 months follow-up in all the patients. All valve protheses switched freely; no tricuspid regurgitation was observed. The results of the present study demonstrate that the CMP-IV is safe and effective in eliminating AF in dextrocardia patients concomitant with valvular surgery.
Introduction
Dextrocardia is a rare, congenital cardiac malformation in which the axis of the heart is indexed to the right side of the thoracic cavity. Dextrocardia with situs inversus totalis refers to all visceral organs, including the heart, being mirrored and is extremely rare1,2. Atrial fibrillation (AF) is the most common arrhythmia that affects millions of people and causes substantial morbidity and mortality, especially with respect to the increased risk of stroke3.
A literature survey was unable to demonstrate a definitive approach for the concomitant Cox-Maze procedure (CMP) and valve surgery in dextrocardia with situs inversus. There are only a few reports of mitral valve replacement in cases of dextrocardia with situs solitus and even less in situs inversus4,5,6,7,8,9. However, those strategies are not applicable to tricuspid valve surgery, not to mention the complex CMP-IV in dextrocardia.
This paper reports the surgical techniques and experience in three cases concerning the biatrial lesion set of the CMP-IV operation using cryoablation or radiofrequency ablation concomitantly with valve surgery in situs inversus dextrocardia. All surgeries succeeded in the maintenance of sinus rhythm (SR) at 12 months follow-up to each patient and at 48 months follow-up to the previous two. Electron microscopy was used to investigate the transmurality of the atrial cryoablation.
CASE PRESENTATION:
Patients and preoperative examination
From February 2016 to September 2020, a 48-year-old female patient, a 55-year-old male patient, and a 39-year-old male patient were successively admitted to the cardiac center with similar complaints of palpitation, dyspnea, and easy fatigability on exertion lasting for years (Table 1). They all denied any familiar history of situs inversus dextrocardia or other cardiac health comorbidities. All patients were referred to electrocardiography (ECG), chest roentgenography (Figure 1), computed tomography (CT), and Doppler transthoracic echocardiography (TTE) routinely for preoperative examination.
Case 1: A 48-year-old woman presented with complaints of palpitation, dyspnea, and easy fatigability on exertion lasting for years. The anamnesis was unremarkable. During the physical examination, a grade 3 systolic blowing murmur was heard in the fifth intercostal space outside the midline of the right clavicle. X-ray plain film and CT scan showed that the contour of the dextrocardia was enlarged and situs inversus totalis. Transesophageal echocardiography revealed moderate to severe mitral regurgitation and mild tricuspid regurgitation after moderate mitral valve prolapse and tricuspid annular dilation. There was no thrombosis, and the diameter of the left atrium was 5.3 cm. AF with a rapid ventricular rate was detected by ECG.
Case 2: A 55-year-old man presented with similar symptoms for years. There was no history of cardiac health comorbidities; however, he had had a stroke about six months ago. On physical examination, a diastolic blowing murmur was heard at the left second intercostal space, radiating to the neck. Plain X-ray and CT scan revealed dextrocardia with an enlarged contour and situs inversus totalis. Transesophageal echocardiography showed moderate aortic valve regurgitation and mild to moderate tricuspid regurgitation following mild aortic prolapse and tricuspid annular dilation. The diameter of the left atrium was 4.5 cm without atrial thrombosis. AF with a rapid ventricular rate was detected by ECG.
Case 3: A 39-year-old male presented with progressive exertional dyspnea and intermittent palpitation without any history of situs inversus dextrocardia or other cardiac health complications. During the physical examination, a grade 3 systolic blowing murmur was heard in the fifth intercostal space outside the midline of the right clavicle. X-ray plain film and CT scan revealed that the contour of dextrocardia was enlarged and situs inversus totalis. Transesophageal echocardiography showed severe mitral valve regurgitation and tricuspid regurgitation after annular enlargement. There was no atrial thrombosis, and the left atrium diameter was 5.8 cm. A 24 h ambulatory ECG demonstrated paroxysmal AF with a total load of 165 min.
Diagnosis, assessment, and plan
Case 1: A biatrial cryo-Cox-Maze IV procedure (cryo-CMP-IV) with mechanic mitral valve replacement and tricuspid annuloplasty were simultaneously performed.
Case 2: A biatrial cryo-CMP-IV procedure with concomitant mechanic aortic valve (AV) replacement and tricuspid annuloplasty was performed.
Case 3: A biatrial CMP-IV procedure, mitral valvuloplasty, and tricuspid annuloplasty were performed simultaneously.
Protocol
Representative Results
Discussion
Dextrocardia is a group of rare heart defects in which the heart is situated on the right side of the thoracic cavity instead of on the left side. One-third of all dextrocardia cases are mirror-image cases, which means the orientation of the cardiac chambers is a mirror image to levocardia (normally situated heart)12. It is estimated that situs inversus dextrocardia associated with situs inversus totalis occurs with an incidence of much less than 1 in 10,000-50,000 births1<…
Disclosures
The authors have nothing to disclose.
Acknowledgements
We thank the patients for participating in this study. We are also grateful to the Biomedicine Electron Microscopy Laboratory of Basic Medical Science School of Central South University, particularly to Xiaoying Wu and Jin Li for technical support. This work was supported by the National Key Research and Development Program (No. 2018YFC1311204).
Materials
| CryoICE | AtriCure, Cincinnati, Ohio | CRYO2 | Cryoablation in case 1 and case 2 was performed exclusively using it with a flexible 10cm in length metal cryoprobe. |
| Medtronic Open Pivot Standard mechanical MV | Medtronic, Minneapolis, Minn | 709291 / MHV 500DM27 STD MITRAL | A 27-mm MV was adopted in case 1. |
| Medtronic Open Pivot Standard mechanical AV | Medtronic, Minneapolis, Minn | 646871 / MHV 500FA23 STD AORTIC | A 23-mm AV was adopted in case 2. |
| bipolar radiofrequency pen | AtriCure Inc., Cincinnati, OH | Maze-IV in case 3 | |
| bipolar radiofrequency clamps | AtriCure Inc., Cincinnati, OH | Maze-IV in case 3 | |
| Goretex | W.L. Gore & Associates, Inc., Elkton, Maryland | A surgical suture made of polytetrafluoroethylene. | |
| rigid mitral ring | Kingstron Bio, Suzhou, China | Element Force ARM32 | A 32 mm ring was adopted in case 3. |
| Tricuspid Sovering Band | Sorin Group Italia S.r.l., VC, Italy | SBG0730 / SB30T | A 30 mm ring was adopted in case 1. |
| Tricuspid Sovering Band | Sorin Group Italia S.r.l., VC, Italy | SQB0240 / SB30T | A 30 mm ring was adopted in case 2. |
| Tricuspid Sovering Band | Sorin Group Italia S.r.l., VC, Italy | SBF0930 / SB28T | A 28 mm band was adopted in case 3. |
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