|Year : 2015 | Volume
| Issue : 4 | Page : 112-115
A Case of Mirror-image Dextrocardia and Situs Inversus Totalis Associated with Tetralogy of Fallot with Patent Ductus Arteriosus with Normal L-loop, Atrioventricular and Ventriculo-arterial Concordance, and Right Aortic Arch: Main Surgeon at Assistant Side
Ram Chandra Sherawat, Anil Sharma, Sunil Dixit, Amit Sharan, Sunil Sample
Department of CTVS, Sawai Man Singh Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||21-Dec-2015|
Ram Chandra Sherawat
E-1/A, Hari Nagar, Shastri Nagar, Jaipur - 302 016, Rajasthan
Source of Support: None, Conflict of Interest: None
Although statistically people with dextrocardia and situs inversus totalis have fewer chances of any congenital cardiac abnormalities; however, its association with tetralogy of fallot (TOF) and patent ductus arteriosus is reported only rarely and can present technical challenges to intracardiac repair. This combination of anomalies is extremely unusual. We report a case of successful surgical treatment of TOF with dextrocardia and situs inversus totalis in a 35-year-old male, and describe the patient's postoperative course, and review the surgical literature relevant to these combined conditions with unbelievable presentation history.
Keywords: Dextrocardia with situs solitus totalis, patent ductus arteriosus, tetralogy of fallot
|How to cite this article:|
Sherawat RC, Sharma A, Dixit S, Sharan A, Sample S. A Case of Mirror-image Dextrocardia and Situs Inversus Totalis Associated with Tetralogy of Fallot with Patent Ductus Arteriosus with Normal L-loop, Atrioventricular and Ventriculo-arterial Concordance, and Right Aortic Arch: Main Surgeon at Assistant Side. Heart India 2015;3:112-5
|How to cite this URL:|
Sherawat RC, Sharma A, Dixit S, Sharan A, Sample S. A Case of Mirror-image Dextrocardia and Situs Inversus Totalis Associated with Tetralogy of Fallot with Patent Ductus Arteriosus with Normal L-loop, Atrioventricular and Ventriculo-arterial Concordance, and Right Aortic Arch: Main Surgeon at Assistant Side. Heart India [serial online] 2015 [cited 2020 Jul 15];3:112-5. Available from: http://www.heartindia.net/text.asp?2015/3/4/112/172356
| Introduction|| |
Though repair in dextrocardia with congenital lesion can be approached from the right side but it's easier to establish cardiopulmonary bypass (CPB) and repair from the left side so during the surgical repair main surgeon stands on the assistant side on the operating table. CPB could be established according to the standard technique with aortic and venous dual drainage of right atrium (RA). Patent ductus arteriosus (PDA) ligation should be done just before going on CPB. There can be technical difficulties during CPB and repair but RA approach lead to successful ventricular septal defect (VSD) patch repair. The right ventricular (RV) outflow tract obstruction can be relieved with use of a transannular patch of pericardium also relieving the pulmonary stenosis (PS) with valvotomy. RA approach seems much sounder than RV for such type of complex congenital lesions with dextrocardia.
| Case Report|| |
A 35-year-old male presented as a diagnosed case of dextrocardia and complete situs inversus. Dextrocardia and situs inversus were confirmed on chest radiograph, electrocardiograph, abdominal ultrasound, and computerized axial tomography of cardiac chambers in the patients. Echocardiography established atrioventricular and ventriculo-arterial concordance with cardiac symptoms. The authors review their experience with dextrocardia and discuss the useful clinical points that aid in evaluating complex anatomy and surgical mire in such type of cases.
This case was presented with complaint of shortness of breath and cyanotic spells since birth but did not take any treatment for such ailment. Later, at the age of 34 years, he was accidentally diagnosed as tetralogy of fallot (TOF) with dextrocardia and situs inversus, when he was taking treatment for brain abscess [Figure 1]. Although, throughout his life, he had experienced decreased exercise tolerance with frequent episodes of cyanosis and squatting, and substantial shortness of breath upon exertion. There was no history of chest pain or palpitations. The patient's medical history was, otherwise, not significant except for brain abscess, when he was 34-year-old. Because of limited access to medical care, he had not undergone medical therapy or surgical treatment for his cardiac condition. The patient was married for last 10 years and had two offspring.
In December 2014, the patient was presented to cardiothoracic and vascular surgery outpatient department as diagnosed case of TOF with brain abscess. His body weight was 55 kg, and his height was 5'6". The physical examination revealed marked cyanosis, scleral hyperemia; grade 2 clubbing and right testicle were lower then left. On percussion, gastric tympany on the right side, hepatic dullness on the left, and cardiac dullness on the right side. The patient's hematocrit on presentation was 60.1%. Cardiac auscultation revealed a grade 3/6 systolic ejection murmur, best heard at the right upper sternal border and radiating to the axillae and back. The results of a pulmonary examination were within normal limits, and no organomegaly was detected. A 12-lead electrocardiogram yielded results consistent with dextrocardia, inverted P waves in leads I and aVL, and an upright P wave in lead aVR [Figure 2]. Chest radiographs were suggestive of dextrocardia with total situs inversus, and a heart size within normal limits [Figure 3]. Transthoracic echocardiograms revealed the anatomy of dextrocardia with situs inversus and TOF with PDA a large cono-VSD and severe RV outflow tract obstruction [Figure 4], normal caliber branch pulmonary arteries, and a small aortopulmonary collateral vessel [Figure 5].
|Figure 4: Two-dimensional echo shows ventricular septal defect and pulmonary stenosis|
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|Figure 5: Computed tomography pulmonary angiography shows the left side inferior vena cava, superior vena cava and right atrium|
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Preoperatively, the patient's heart rate was 110 beats/min, his blood pressure was 102/62 mmHg, and his transcutaneous oxygen saturation was 82% on room air during the surgical repair main surgeon on the assistant side on the operating table. Though repair in dextrocardia with congenital lesion can be approached from the right side but it's easier to establish CPB and repair from the left side. CPB was established according to the standard technique with aortic and venous dual drainage of RA. PDA ligation was done just before going on CPB. VSD repair was carried out through right atriotomy situated on the left side with GORE-TEX® patch. There was a technical difficulty during CPB and repair but thereafter RA approach led to successful VSD patch repair [Figure 6]. The RV outflow tract obstruction was relieved with the use of a transannular patch of pericardium also relieving the PS with valvotomy. RA approach seems much sounder than RV for such type of complex congenital lesions with dextrocardia. The patient's postoperative course was uneventful. The patient was discharged from the hospital on 7 th postoperative day, on furosemide and spironolactone therapy.
|Figure 6: Intraoperative picture showing position of opened right atrium|
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| Discussion|| |
Dextrocardia is a congenital cardiac malrotation in which the heart is situated on the right side of the body (dextroversion) with the apex pointing to the right. Dextrocardia is believed to occur in approximately 1 in 12,000 people. 
Situs inversus (also called situs transversus or oppositus) is a congenital condition in which the major visceral organs are reversed or mirrored from their normal positions. The normal arrangement is known as situs solitus. In other rare cases such as situs ambiguous, or heterotaxy, and situs cannot be determined. Situs inversus is thought to be present in 0.01% of the population, or a 1 in 10,000 chance. 
Dextrocardia with situs inversus refers to the heart being a mirror image situated on the right side. For all visceral organs to be mirrored, the correct term is dextrocardia situs inversus totalis.
The incidence rate in the general population is estimated at 1/8000-1/25,000.  The heart and the thoracic and abdominal viscera are mirror images of normal. The bronchi are inverted with the morphologic right bronchus concordant with the morphologic RA and the trilobed lung and with the morphologic left bronchus concordant with the morphologic left atrium and the bilobed lung. The heart is right sided, and the right hemidiaphragm is lower than the left hemidiaphragm. The descending aorta is on the right; the ascending aorta, aortic knuckle, and pulmonary trunk are their mirror image position; and the anatomic right ventricle lies to the left of the anatomic left ventricle (L-bulboventricular loop), which is normal for situs inversus just as a D-bulboventricular loop is normal for situs solitus.
Although statistically people with dextrocardia situs inversus do not have any medical problems from the disorder, they may be prone to a number of bowel, esophageal, bronchial, and cardiovascular disorders (such as double outlet right ventricle, endocardial cushion defect, and PS). Certain cardiovascular and pulmonary disorders related to dextrocardia can be life-threatening if left unchecked.
TOF is diagnosed in approximately 3.5% of patients who have congenital heart defects. The condition consists of VSD, overriding aorta, RV outflow tract obstruction, and RV hypertrophy.
Although TOF is a common congenital anomaly reports of its association with dextrocardia and situs inversus are few. In 1952, Scragg and Denny reported the first documented case of TOF with situs inversus,  in a 2½-year-old girl who presented with prolonged cough, failure to thrive, marked cyanosis, and clubbing in the presence of unrepaired TOF.
The reported prevalence of the three anomalies together is low. In 147 adults with TOF, two patients had dextrocardia (prevalence, 1.4%).  A study of 63 patients with dextrocardia yielded one patient with TOF and situs inversus (prevalence, 1.6%).  The outcomes of treating congenital cardiac anomalies in patients with dextrocardia depend on the complexity of the cardiac lesions. Bohun et al.  reported a 91% survival rate after at least 2 years in patients with complex congenital heart disease and dextrocardia; all the patients who died had complex cardiac anatomy with more than one malformation. After the initial diagnosis of dextrocardia, physicians can treat patients with TOF and dextrocardia as they would any patient with TOF, with the use of similar surgical procedures and postoperative monitoring.
The altered spatial orientation of the cardiac structures can present a challenge in patch closure of the VSD. The surgeon approaches the VSD at a different angle in a patient with dextrocardia than in a patient with levocardia [Figure 5].
Dextrocardia with situs invesus is characterized by a right thoracic heart, a right-sided stomach; a left-sided liver, a left-sided morphologic right bronchus and trilobed lung, a right-sided morphologic left bronchus and bilobed lung, and inverted positions of the atria. Dextrocardia and situs inversus (mirror image) are likely to be discovered incidentally on a routine chest X-ray, especially when the heart is structurally normal, as is usually the case. On physical examination, gastric tympany and cardiac dullness are on right, hepatic dullness is on the left, and heart sounds are louder on the right side of the chest. The X-ray confirms the stomach bubble on the right, the liver on the left, and cardiac silhouette to the right of midline. The electrocardiogram shows an inverted P wave, negative QRS complex and inverted T wave in lead I, reversal of the QRS pattern in lead aVR and lead aVL, and reversal of corresponding right and left precordial leads. Two-dimensional echo visualizes the inferior vena cava and the liver to the left of the spine and the stomach and descending aorta to the right of the spine.
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| References|| |
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Douard R, Feldman A, Bargy F, Loric S, Delmas V. Anomalies of lateralization in man: A case of total situs inversus. Surg Radiol Anat 2000;22:293-7.
Scragg JN, Denny M. Dextrocardia, tetralogy of Fallot, and situs inversus; report of a case. S Afr Med J 1952;26:1025-8.
Abraham KA, Cherian G, Rao VD, Sukumar IP, Krishnaswami S, John S. Tetralogy of Fallot in adults. A report on 147 patients. Am J Med 1979;66:811-6.
Evans WN, Acherman RJ, Collazos JC, Castillo WJ, Rollins RC, Kip KT, et al.
Dextrocardia: Practical clinical points and comments on terminology. Pediatr Cardiol 2010;31:1-6.
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6]