Bolz, D.

Dodge-Khatami, A.

Knirsch, W.

Valsangiacomo Büchel, E.

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A newborn was referred to our hospital because ofprominent cardiac pulsations in the subxyphoid regionassociated with supraumbilical midline cutaneous le-sions (Fig. 1). Echocardiography revealed mesocardiawith a ventricular septal defect, a secundum atrialseptal defect, and an unroofed coronary sinus. Themorphological correlate for the pulsatile tumor was aleft ventricular diverticulum diagnosed by transtho-racic echocardiography (Fig. 2). Cardiac magneticresonance imaging (MRI) confirmed the presence of aleft ventricular diverticulum originating from the apexof the heart and ruled out the presence of a dia-phragmatic hernia (Fig. 3).Cardiac surgical repair, performed at 4 weeks of age,confirmed the preoperative findings (Fig. 4). The leftventricular diverticulum was a continuation of the apexof the left ventricle and was totally covered by peri-cardium. We found a midline muscular defect of theabdominal wall and a missing linea alba, as well as amissing xyphoid, but no evidence of diaphragmatichernia or additional midline malformations.This represents a forme fruste of Cantrell’s Syn-drome. Cantrell’s pentalogy includes a midline, sup-raumbilical abdominal wall defect, a defect of thelower sternum, a deficiency of the anterior diaphragm,a defect in the diaphragmatic pericardium, and con-genital intracardiac defects [1]. In this case, the defi-nition of the topographic localization of thediverticulum performed by MRI and of its relation tothe diaphragm was essential in planning the surgicalrepair.References1. Cantrell JR, Haller JA, Ravitch MM (1958) A syndrome ofcongenital heart defects involving the abdominal wall, ster-num, diaphragm, pericardium and heart. Surg Gynecol Obstet107:602–614W. Knirsch (&) Æ E. V. Bu¨chelDivision of Pediatric Cardiology, University Children’sHospital Zurich, Steinwiesstr-75, CH 8032, Zurich,SwitzerlandE-mail: walter.knirsch@kispi.unizh.chA. Dodge-KhatamiDivision of Cardiovascular surgery, University Children’sHospital Zurich, SwitzerlandD. BolzDivision of Pediatric Cardiology, University Children’sHospital Basel, SwitzerlandPediatr Cardiol (2006) 27:652–654DOI 10.1007/s00246-005-6001-2123IMAGES IN PEDIATRIC CARDIOLOGYCantrell’s Syndrome Forme Fruste in a Newborn Diagnosed byTransthoracic Echocardiography and Cardiac MagneticResonance ImagingW. Knirsch Æ A. Dodge-Khatami Æ D. Bolz ÆE. Valsahgiacomo Bu¨chel Springer Science+Business Media, Inc. 2005Fig. 1 Preoperative status showing thesubxyphoid-to-supraumbilical coveredmidline defect of the abdominal wallFig. 2 (Left) Two-dimensionalechocardiogram of the left ventriculardiverticulum (D, arrows) and small leftventricle (LV). (Right) Color Dopplershowing blood flow (red) into the diverticulum(D)Pediatr Cardiol (2006) 27:652–654 653123Fig. 4 Intraoperative situs of the leftventricular diverticulum (D). LV, leftventricle, RV, right ventricleFig. 3 Contrast-enhaced MR angiography demonstrating theleft ventricular diverticulum (D) with ‘‘vermiform’’ structureoriginating from the apex of the left ventricle (LV) and itsrelationship to the diaphragm (asterists) (lateral projection)654 Pediatr Cardiol (2006) 27:652–654123

Cantrell's Syndrome Forme Fruste in a Newborn Diagnosed by Transthoracic Echocardiography and Cardiac Magnetic Resonance Imaging

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Cantrell's Syndrome Forme Fruste in a Newborn Diagnosed by Transthoracic Echocardiography and Cardiac Magnetic Resonance Imaging

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