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Hypoplastic Left Heart Syndrome With Dextrocardia and Situs Solitus Marcin Szczechowicz, Katarzyna Januszewska, Christian Jux and Edward Malec World Journal for Pediatric and Congenital Heart Surgery 2014 5: 323 DOI: 10.1177/2150135113515277 The online version of this article can be found at: http://pch.sagepub.com/content/5/2/323

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Case Report

Hypoplastic Left Heart Syndrome With Dextrocardia and Situs Solitus

World Journal for Pediatric and Congenital Heart Surgery 2014, Vol. 5(2) 323-325 ª The Author(s) 2013 Reprints and permission: sagepub.com/journalsPermissions.nav DOI: 10.1177/2150135113515277 pch.sagepub.com

Marcin Szczechowicz, MD1, Katarzyna Januszewska, MD, PhD1, Christian Jux, MD, PhD1, and Edward Malec, MD, PhD1

Abstract Hypoplastic left heart syndrome with dextrocardia and situs solitus is a very rare condition. We describe a 3.5-year-old male child with this constellation who underwent multistage repair and a successful extracardiac Fontan operation in our hospital. Keywords hypoplastic left heart syndrome, Fontan, cardiac anatomy/pathologic anatomy, cardiac malposition Submitted July 23, 2013; Accepted November 6, 2013.

Introduction Dextrocardia with situs solitus coexisting with hypoplastic left heart syndrome (HLHS) is very uncommon. We describe our case and as far as possible try to explain this situation.

Case Report A 3.5-year-old boy was admitted to our hospital in order to perform Fontan completion. He was prenatally diagnosed with HLHS (mitral valve atresia and aortic valve atresia) with dextrocardia and situs solitus. In the echo examination, a four-chambered heart with significant dominance of the right side was shown. The cardiac apex and stomach were on opposite sides (dextrocardia) and the left ventricle was small and akinetic, with hyperechogenic endocardium. The ventricular septum was intact, and there was absent left atrioventricular connection (mitral atresia) and single pulmonary outlet (aortic valve atresia). There was a concordant cardiac loop (d-loop), corresponding to the visceroatrial situs and to the locations of the great arteries {S, D, S}. The atretic aortic valve was 2 mm in diameter. The ascending aorta was similarly diminutive. A left-to-right shunt was visible at the level of patent foramen ovale. The systemic and pulmonary venous returns were normal. The right ventricular ejection fraction was 45%, and the rhythm was normal sinus. A Norwood procedure was performed on day 12 of life, with a 5-mm right ventricle to pulmonary artery (RV-PA) conduit as the source of pulmonary blood flow. After six months, he underwent replacement of the RV-PA conduit with a 6-mm diameter polytetrafluoroethylene tube in another institution because of the presence of hypoplastic pulmonary arteries that was felt to be a contraindication to the performance of a

standard cavopulmonary connection. At 2.5 years of age, a superior cavopulmonary connection, with removal of the RVPA conduit, was successfully performed in our center. At the time of admission for the completion Fontan procedure at age 3.5 years, the only symptoms were slight perioral cyanosis with intensive exertion, which had become more perceptible within the previous two to three months. There were bilateral epicanthi and a short, wide neck. The chest x-ray confirmed the presence of dextrocardia with situs solitus of the abdominal viscera (Figure 1). The electrocardiogram findings were typical for right ventricular hypertrophy, and the amplitude of the R-waves decreased from V1 to V5, with no conduction or rhythm disturbances. The admission echocardiogram (Figure 2) demonstrated dextrocardia with good systolic function of the systemic morphologically RV. There was no tricuspid valve insufficiency, and there was mild neoaortic valve insufficiency. The inferior vena cava was on the right side, and the abdominal aorta was leftward and anterior to the vertebral column. Distinct dilatation of the neoaorta was demonstrated. Abdominal ultrasonography revealed no significant pathology, with only one small accessory spleen being demonstrated. The liver was on the right side. The pre-Fontan cardiac catheterization demonstrated a mean systemic venous and PA pressure of 15 mm Hg, transpulmonary gradient of 4 to 5 mm Hg, pulmonary capillary wedge pressure of 10 to 11 mm Hg, and inferior

1

Pediatric Cardiac Surgery, Universita¨tsklinikum Mu¨nster, Mu¨nster, Germany

Corresponding Author: Marcin Szczechowicz, Pediatric Cardiac Surgery, Universita¨tsklinikum Mu¨nster, Albert-Schweitzer-Campus 1, Geba¨ude: A1, 48149 Mu¨nster, Germany. Email: [email protected]

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World Journal for Pediatric and Congenital Heart Surgery 5(2)

Figure 2. Echocardiography. Hypertrophied right ventricle and small hypoplastic left ventricle.

Figure 1. Chest x-ray at admission. Cardiac axis-directed rightward, left aortic arch, normal bronchial, and visceral pattern.

vena cava pressure of 8 mm Hg. The end-diastolic pressure in the systemic RV was 11 to 12 mm Hg. There was no restriction at the level of the interatrial communication. Some venous systemic-to-pulmonary collaterals were noted, and these were occluded with coils. The aortic arch was left sided. A significant stenosis of the proximal right PA between the Glenn anastomosis and stump of the main PA required balloon angioplasty. A nonfenestrated extracardiac completion Fontan was performed on a beating heart with normothermic cardiopulmonary bypass. Postoperatively no complications occurred, and the child was discharged from hospital on postoperative day 11, with clinical signs of good cardiac output and an arterial oxygen saturation of 95%.

Discussion A congenital variation in the heart position with the cardiac base-to-apex axis directed rightward is termed dextrocardia. In every case, the patient should be precisely diagnosed, because additional cardiac and extracardiac malformations are often present. It is important to analyze the relative locations of the morphologically right and left atria and of the morphologically right and left ventricles and also the arrangement of the other thoracic and abdominal organs. These patients may be more difficult to assess than patients with the usual position and orientation of the heart.1 The visceral situs almost always corresponds to the atrial situs. In situs solitus, with a right-sided liver and a left-sided stomach, the inferior vena cava is situated to the right side of the spine and connects to the morphologically right

atrium, situated in the right side of the chest. The opposite situation occurs in situs inversus. Situs ambiguous means an abnormal symmetry of the body, with either two morphologically left, two right, or two undetermined atria.1,2 Looping of the bulboventricular part of the heart tube is strongly correlated with situs. In situs solitus, d-looping is most common; conversely, in situs inversus an l-looped heart is typical.2 In a normally developing heart, with situs solitus and d-looping, during the first stages of fetal life the apex of the heart is located on the right side of the chest but at the end of the first month of prenatal development moves to the left side. In hearts with situs inversus and l-looping, conversely, the leftdirected apex moves to the right side. Dextrocardia may therefore result from normal development of l-looped heart in situs inversus or incomplete shift of the cardiac apex to the left in a d-looped heart. Also in rare cases, the type of looping (either d or l) cannot be established.1,2 Our patient had no laterality disorders. Accessory spleen tissue, found on ultrasonography, does not meet the definition of heterotaxia or polysplenia syndrome and is a common finding, occurring in 10% to 30% of individuals in general population.3,4 The exact incidence of true dextrocardia is not well known, but this condition reportedly occurs in 0.0083% to 0.01% of live births and is often accompanied by other cardiac malformations, such as transposition of great arteries, doubleoutlet ventricle, single ventricle, anomalous pulmonary venous return, and others.1,2,5,6 Situs solitus occurs in about 33% to 34% of the dextrocardia cases5,6; however, we did not find any report of HLHS in situs solitus and dextrocardia. Development of congenital cardiac defects in humans is still not well understood. There are some genetic abnormalities described, resulting in specific patterns of pathologic development that are more often met in certain heart malformations. Mendelian and chromosomal syndromes can be found in approximately 20% of the cases. In the remaining 80%, the

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causes and molecular mechanisms are unclear.7 Dextrocardia with situs solitus occurs in about 30 newborns per million. Hypoplastic left heart syndrome is also a relatively rare condition, occurring in 154 to 279 cases per million.8 Using simple multiplication, a crude approximation of the incidence of this unusual cardiac malformation is estimated at 5 to 10 cases in 1  109 newborns. That is likely, though, only if the inheritance of HLHS is independent from dextrocardia with situs solitus, and if all fetuses, so affected, were live births. In a patient with dextrocardia with the inferior vena cava on the right side, some difficulties could be expected during the Fontan operation, because the anastomosis of the extracardiac conduit to the inferior vena cava must be made behind the cardiac apex. Theoretically, the chances of compression of the conduit may be increased in this setting. While some surgeons choose to use a ring-reinforced conduit to address this situation, there is no evidence that this is essential to produce a successful surgical result. For the same reason, the Fontan operation with lateral tunnel could be recommended in patients with dextrocardia and right-sided inferior vena cava. The potential advantage of this method is elimination of the possibility of compression of an extracardiac conduit by the heart. A potential disadvantage is the awkward and relatively inaccessible location of the systemic venous atrium behind the ventricular mass. There was, however, no need to use either of these techniques in our patient, and the Fontan operation with a nonreinforced extracardiac conduit was performed with good outcome. During the operation, gentle rotation and elevation in the heart are required to expose the inferior vena cava for the inferior anastomosis. This, however, is usually not a problem on cardiopulmonary bypass.

Declaration of Conflicting Interests The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

Funding The author(s) received no financial support for the research, authorship, and/or publication of this article.

References 1. Van Praagh R, Van Praagh S, Vlad P, Keith J. Anatomic types of congenital dextrocardia. Diagnostic and embryologic implications. Am J Cardiol. 1964;13: 510-531. 2. Maldijan PD, Saric M. Approach to dextrocardia in adults: review. Am J Roentgenol. 2007;188(6 suppl): S39-S349. 3. Peeters H, Devriendt K. Human laterality disorders. Eur J Med Genet. 2006;49(5): 349-362. 4. Yildiz AE, Ariyurek MO, Karcaaltincaba M. Splenic anomalies of shape, size, and location: pictorial essay. ScientificWorldJournal. 2013;2013: 321810. 5. Evans WN, Acherman RJ, Collazos JC, et al. Dextrocardia: practical clinical points and comments on terminology. Pediatr Cardiol. 2010;31(7): 1-6. 6. Lev M, Liberthson RR, Friedrich Eckner A, Arcilla RA. Pathologic anatomy of dextrocardia and its clinical implications. Circulation. 1968;37(6): 979-999. 7. Bentham J, Bhattacharya S. Genetic mechanisms controlling cardiovascular development. Ann N Y Acad Sci. 2008;1123: 10-19. 8. Hoffman J, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol. 2002;39(12): 1890-1900.

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Hypoplastic left heart syndrome with dextrocardia and situs solitus.

Hypoplastic left heart syndrome with dextrocardia and situs solitus is a very rare condition. We describe a 3.5-year-old male child with this constell...
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