A Rare Presentation of Right Lung Hypoplasia Associated with
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A Rare Presentation of Right Lung Hypoplasia Associated with
OLGU SUNUMU • CASE REPORT Koşuyolu Kalp Dergisi 2012;15(2):80-83 A Rare Presentation of Right Lung Hypoplasia Associated with Dextrocardia and Visceral Malposition Sağ Akciğer Hipoplazisi, Dekstrokardi ve Viseral Malpozisyonu Olan Nadir Bir Olgu Sunumu Ayşe Yıldırım1, Zülal Uslu2, Aysu Türkmen Karaağaç3, Füsun Güzelmeriç4, Ayşe Baysal4 1 1 2 2 3 3 4 4 Department of Pediatric Cardiology, Kosuyolu Heart Center, Kartal, Istanbul, Turkey Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi, Pediatrik Kardiyoloji Bölümü, İstanbul, Türkiye Department of Radiology, Kosuyolu Heart Center, Kartal, Istanbul, Turkey Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi, Radyoloji Bölümü, İstanbul, Türkiye Department of Pediatry, Kosuyolu Heart Center, Kartal, Istanbul, Turkey Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi, Pediatri Bölümü, İstanbul, Türkiye Department of Anesthesia and Reanimation, Kosuyolu Heart Center, Kartal, Istanbul, Turkey Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi, Anestezi ve Reanimasyon Bölümü, İstanbul, Türkiye ABSTRACT Lung hypoplasia is often associated with pulmonary venous return abnormalities, referred to as the Scimitar syndrome, in pediatric patients. A two day-old male patient presented to our clinic with respiratory distress and mild cyanosis. Diagnostic studies revealed dextrocardia, right sided hypoplasia of upper and middle lung lobes and enlargement of the left lung due to the compensation, midline liver, right sided stomach and right sided spleen. No pulmonary venous return abnormalities were detected. This is the first report of lung hypoplasia associated with heterotaxy, visceral malposition and normal pulmonary venous return. Key Words: Abnormalities; infant, newborn; disease. Received: 29.04.2011 l Accepted: 09.06.2011 Yazışma Adresi/ Correspondence Dr. Ayşe Yıldırım Kartal Koşuyolu Yüksek İhtisas Eğitim ve Araştırma Hastanesi, Pediatrik Kardiyoloji Bölümü, Denizer Caddesi, Cevizli Kavşağı No: 2 34846 Cevizli, Kartal, İstanbul-Türkiye e-posta [email protected] 80 ÖZET Pediatrik hastalarda akciğer hipoplazisine genellikle pulmoner venöz dönüş anomalisi eşlik eder ve bu olgular Scimitar sendromu olarak bilinir. Kliniğimize iki günlük erkek bir bebek solunum sıkıntısı ve hafif siyanozla getirildi. Bu hastada dekstrokardi, sağ akciğer üst ve orta loblarda hipoplazi, sol akciğer genişlemesi, sağ yerleşimli mide ve dalak tespit edildi. Diğer akciğer hipoplazili olgulardan farklı olarak bu hastada pulmoner venöz dönüş anomalisi yoktu. Bu olgu, akciğer hipoplazisi, heterotaksi, viseral malpozisyon ve normal venöz dönüşü olması nedeniyle sunulmuştur. Anahtar Kelimeler: Anormallikler; bebek, yenidoğan; hastalık. Geliş Tarihi: 29.04.2011 l Kabul Tarihi: 09.06.2011 Kosuyolu Kalp Derg 2012;15(2):80-83 l doi: 10.5578/kkd.3432 Yıldırım A, Uslu Z, Türkmen Karaağaç A, Güzelmeriç F, Baysal A. IntroductIon Situs anomalies are rare, complex and confusing. Situs inversus refers to an anatomic arrangement that is mirror image of situs solitus. Situs ambigious or heterotaxy refers to malposition of the thoraco-abdominal organs and vessels including indeterminate atrial arrangement and, complex congenital heart diseases(1). The two major subcategories of heterotaxy are heterotaxy with polysplenia and heterotaxy with asplenia. Heterotaxy with asplenia has often been described as bilateral right-sidedness (right atrial isomerism), bilateral trilobed lungs and bilateral epiarterial bronchi. Heterotaxy with polysplenia is bilateral left sidedness (left atrial isomerism), two lobe lungs and hypoarterial bronchi(2,3). With respect to the congenital heart diseases, lung hypoplasia is frequently associated with the pulmonary venous return anomaly referred to as the Scimitar syndrome. This syndrome is described as the partial anomalous pulmonary venous drainage of right lung into the inferior vena cava (IVC), partial systemic arterial blood supply, and hypoplasia of the affected lung, with bronchial abnormalities and abnormal lobation(4). This case report includes a newborn with dextrocardia, patent ductus arteriosus, pulmoner atresia, atrial septal defect, ventricular septal defect, transposition of great arteries and visceral malposition. In contrast to the previous reports of asplenia and bilateral trilobed lungs in right lateralization, this patient had a right-sided spleen, hypoplastic right lung and a two-lobed left lung. This is a unigue presentation due to the absence of pulmonary venous return abnormalities associating with the visceral malposition and the complex cardiac pathologies. care unit. In his chest X-ray, he had dextrocardia and a large shadow of the thymus. The oxygen saturation was 80-85%. The diagnosis of heterotaxy syndrome and right atrial isomerism was initially based on a suspicion, after viewing juxtaposed aorta and IVC on the same side on the patient’s echocardiography. Other cardiac pathologies detected through the echocardiography included dextrocardia, ventricular septal defect, atrial septal defect, pulmonary atresia, patent ductus arteriosus and transposition of great arteries. Subsequent thoracic and abdominal computerized tomography evaluations of the patient revealed hypoplasia of upper and middle lung lobes in addition to dextrocardia, a midline liver, right-sided spleen, right-sided stomach and enlargement of the left lung due to compensation and normal pulmonary venous return and juxtaposed aorta and IVC (Figures 1-5). The shape and the junction of atrial appendages on computerized tomography images were found bilaterally wide and triangular in shape. During the 15 days of evaluation period, the patient remained hemodynamically stable. He had no failure to thrive. He was fed by his mother’s milk and gained weight after his discharge from the hospital. DISCUSSION Patients with situs ambiguous or heterotaxy syndrome usually present with complex cardiovascular anomalies. In right lateralization or the asplenia syndrome, commonly observed cardiac malformations include common atriventricular canal, univentricular heart, transposition of great arteries, anomalous pulmonary venous return(2). In this case CASE REPORT A two day-old male patient presented to our clinic with respiratory distress. After 38 weeks of normal gestation, a 28 year-old woman had a normal delivery. There was no history of consanguinity or genetic disorders in the family. In the physical examination, his weight was 2700 g, height was 47 cm, he had no major cranial or facial anomaly, he had difficulty in breathing and mild cyanosis with a respiratory rate of 45 breaths per minute and no breath sounds on the right upper and middle lung fields, his heart rate was 125 beats per minute with a continuous murmur under the right clavicula, his liver was 2 cm palpable below the sternum in the midline, his spleen was not palpable and the remaining systems were normal. The cardiac rhythm, oxygen saturation and the blood pressure of the patient was monitorised regularly in the intensive Koşuyolu Kalp Dergisi 2012;15(2):80-83 Figure 1. Black arrow shows hypoplastic right lung, white arrow shows normal lung field (R: Right, L: Left). 81 A Rare Presentation of Right Lung Hypoplasia Associated with Dextrocardia and Visceral Malposition Sağ Akciğer Hipoplazisi, Dekstrokardi ve Viseral Malpozisyonu Olan Nadir Bir Olgu Sunumu Figure 4. Juxtaposition of vena cava inferior (V) and aorta (A) (R: Right, L: Left). Figure 2. Dextrocardia (Dex), patent ductus arteriosus (PDA) and pulmonary arteries (PA) in computerized tomography angiography (L: Left, R: Right). Figure 5. Midline liver, right stomach (S) and spleen (Sp), vena cava inferior (V) (R: Right, L: Left). on of the heart leading to the hypoplasia of the lung might have taken place just after the normal embryological development of the pulmonary veins. Figure 3. White arrows show four pulmonary veins joining to the left atrium. report, right lateralization was accompanied by dextrocardia and the other complex cardiac pathologies. Although there is right upper and middle lobe hypoplasia, pulmonary venous return was normal. We assume that the malpositi- 82 The diagnostic approach to situs abnormalities is based on the investigation of the morphology of the atrial appendage, the venoatrial connection and other visceral disturbances(1,5). Traditionally, echocardiography and angiography provide detailed information on congenital heart diseases, but in the case of heterotaxy syndrome, these modalities have limitations in imaging cardiac, thoracic, and visceral anomalies completely. Computerized tomography has the advantages while visualising the thoracoabdominal organs and vessels(6). In our case we first Koşuyolu Kalp Dergisi 2012;15(2):80-83 Yıldırım A, Uslu Z, Türkmen Karaağaç A, Güzelmeriç F, Baysal A. diagnosed dextrocardia in routine echocardiography and then suspected of heterotaxia syndrome and evaluated the thoracoabdominal organs by computerized tomography angiography to reach the definit diagnosis. Heterotaxy is a class of birth defects in which left and right paterning (L-R) occurs at an early stage of the developing embryo, before organ formation. Unpaired organs of the chest and abdomen begin to develop in the midline and then move to their final asymmetric positions. The etiology of this asymmetric development has not been completely understood. Hereditary transition, incomplete penetrance, single gene mutation, and environmental factors were accused for this asymmetric development(1). In this case report, we believe that a defect in the L-R pattern occurred at an early stage of embryonic development resulting with the heterotaxic presentation. The location of the heart far away from the midline towards the end of the right side of the chest is considered to be the reason of the development of right sided lung hypoplasia, especially at the upper and middle lobes. Therefore, we suggest that all patients who present with dextrocardia should undergo a detailed computerized tomography angiographic study for the evaluation of thoracoabdominal organs and vessels. As different from other heterotaxia cases our patient presented with respiratory distress in addition to dextrocardia. Therefore we suggest that the differential diagnosis of respiratory distress should include lung hypoplasia. In right sided lung hypoplasia, chest X-ray Koşuyolu Kalp Dergisi 2012;15(2):80-83 may not be a useful diagnostic tool as dextrocardia and the shadow of the thymus may form a misleading image. If there is any suspicion, computerized tomography of the chest would be a better choice to determine the presence of lung hypoplasia. We believe that it is crucial to realise the situs abnormalities and their radiological appearances in order to recognize the children with increased risk of congenital heart disease, respiratory distress due to lung hypoplasia and immune deficiency due to asplenia or volvulus with malrotation. REFERENCES 1. Belmont JW, Mohapatra B, Towbin JA, Ware SM. Molecular genetics of heterotaxy syndromes. Curr Opin Cardiol 2004;19:216-20. 2. Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited: imaging of the heterotaxy syndrome. Radiographics 1999;19:83752. 3. Durán MA, Guereña A. Sequence of right laterality with spleen: widening the spectrum of heterotaxy. Pediatr Pathol Mol Med 2002; 21:461-5. 4. Neill CA, Ferencz C, Sabiston DC, Sheldon H. The familial occurrence of hypoplastic right lung with systemic arterial supply and venous drainage “scimitar syndrome. Bull Johns Hopkins Hosp 1960;107:1-21. 5. Van Praagh S. Cardiac malpositions and the heterotaxy syndrome. In: Nadas AS, Fyler DC (eds). Nadas’ Pediatr Cardiol. 2nd ed. Philadelphia: Hanley & Belfus, 2006:675-95. 6. Chen SJ, Li YW, Wang JK, Chiu IS, Su CT, Hsu JC, et al. Three-dimensional reconstruction of abnormal ventriculoarterial relationship by electron beam CT. J Comput Assist Tomogr 1998;22:560-8. 83
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after his discharge from the hospital.
DISCUSSION
Patients with situs ambiguous or he...