Pentalogy of Cantrell: Is Echocardiography Sufficient in the Neonatal Period?

Zani‐Ruttenstock, Elke; Zani, Augusto; Honjo, Osami; Chiu, Priscilla

doi:10.1055/s-0037-1599794

Cited by 4 publications

(4 citation statements)

References 6 publications

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“…Multidetector computerized tomography (MCDT) scanning is also useful for detecting intra-cardiac defects and has the advantages of being faster than magnetic resonance imaging (MRI) as well as avoiding the need for anesthesia and ECG-gating acquisition (16). The use of cardiac MRI is advocated especially in cases of a left ventricular diverticulum, defined as partial ectopia cordis and characterized by a muscular or fibrous fingerlike appendix of the ventricular wall beyond the myocardial margin, where early diagnosis is important to avoid impending complications (17,18).…”

Section: Post Natal Evaluationmentioning

confidence: 99%

“…Cardiac and pericardial defects vary and are repaired accordingly. In the event of a ventricular diverticulum, surgical repair is indicated early in life because of the risks of thrombogenic events, spontaneous or traumatic rupture, and sudden death by tachyarrhythmias (18,28). Some stable intra-cardiac defects, such as an atrial septal defect or a ventricular septal defect may be repaired initially as well.…”

Section: Initial Management and Surgical Repairmentioning

confidence: 99%

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Pentalogy of Cantrell

Williams

Marayati

Beierle

2019

Seminars in Pediatric Surgery

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Pentalogy of Cantrell is a constellation of five congenital defects that pose a unique challenge for surgeons. Defects of the heart, pericardium, diaphragm, sternum, and anterior abdominal wall are pathognomonic. Although the incidence of this defect is small, it is critical to identify it in a timely fashion in order to adequately address all aspects. Early diagnosis, supportive care, and strategic surgical planning with a multidisciplinary team are all key components in managing patients with Pentalogy of Cantrell. In this text we sought to explore the evolution of both the understanding and treatment for this complex entity and provide current recommendations to today's pediatric caregivers.

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Section: Post Natal Evaluationmentioning

confidence: 99%

Section: Initial Management and Surgical Repairmentioning

confidence: 99%

Pentalogy of Cantrell

Williams

Marayati

Beierle

2019

Seminars in Pediatric Surgery

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“…The absence of the lower sternum leads to direct exposure of mediastinal visceral organs to the outside world, allowing them to easily be injured by external forces and even leading to contrary movements. 26 27 However, it is often difficult to repair sternal defects. The aim of the repair of sternal defects is to protect the organs and tissues in the mediastinum, prevent the contrary movement of organs in the mediastinum caused by breathing, and eliminate external abnormalities.…”

Section: Discussionmentioning

confidence: 99%

Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center

Wang

et al. 2021

Eur J Pediatr Surg

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Objective To summarize experience in the treatment of pentalogy of Cantrell (POC) in our hospital and explore the effect of artificial materials in repairing sternal defects. Materials and Methods A retrospective analysis was performed on treatment of five children with POC treated by using the Gore-Tex patch and titanium mesh in the Department of Cardio-Thoracic Surgery, Children's Hospital of Chongqing Medical University, from January 2010 to January 2019. Results The concurrent conditions included double outlet of right ventricle (n = 2), ventricular septal defect (VSD) and atrial septal defect (ASD) (n = 1), VSD and ASD and patent ductus arteriosus (n = 1), and VSD and left ventricular diverticulum (n = 1) in five cases with POC. Color Doppler echocardiography and computed tomography (CT) + three-dimensional (3D) reconstruction of the thorax and abdomen were performed preoperatively. The cardiac malformation was corrected according to color Doppler echocardiography, and a Gore-Tex patch was used to repair the pericardial defect. Titanium mesh was made according to CT 3D reconstruction with a 3D printing mold to repair sternal defects. All patients underwent a one-stage operation, all hearts were eventually repositioned, no deaths occurred after the operation, and follow-up was performed for 6 months to 2 years. The patients recovered well, and the exterior thorax was normal. Conclusion The diagnosis of POC is not difficult. The priority of surgical treatment for POC is to obtain satisfactory corrections of cardiac malformation. The repair of the pericardial defect with the Gore-Tex patch and the sternal defect with the titanium mesh can make the heart return to the mediastinum, reduce the pressure on the heart, reduce the surgical trauma, reduce the difficulty of repairing the sternal defect, and optimally restore the exterior thorax.

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“…The first clinical feature of pentalogy of Cantrell is a cardiac malformation, and echocardiography has become the key to discovering this disease ( 1 , 7 , 8 ). Still, echocardiographers have an insufficient understanding of pentalogy of Cantrell, which may lead to missed diagnoses, especially in cases lacking the most obvious signs ( 9 ). Therefore, the present study presents a case of pentalogy of Cantrell, with a focus on the clinical and imaging findings.…”

Section: Introductionmentioning

confidence: 99%

Clinical and ultrasound findings of pentalogy of Cantrell in a newborn: A case report

et al. 2022

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BackgroundPentalogy of Cantrell is a rare and deadly syndrome, manifesting as intracardiac anomalies and ventricular diverticulum. Echocardiographers have an insufficient understanding of pentalogy of Cantrell, which may lead to missed diagnoses, especially in cases lacking the most obvious signs.Case summaryOne of twin male infants, at a gestational age of 37 weeks, was found with a cardiac murmur and a pulsatile mass in the midline supraumbilical abdomen for 2 days. Echocardiography on admission indicated congenital heart disease. A cardiac murmur was detected in the 3–4 intercostal space and extensively spread. The infant was diagnosed with pentalogy of Cantrell by ultrasound and computed tomography angiography (CTA) preoperatively. The patient underwent heart deformity surgery and was followed up for 16 months. The patient's cardiac structure and function returned to normal.ConclusionIntracardiac anomaly and ventricular diverticulum are the primary manifestations of pentalogy of Cantrell. Pentalogy of Cantrell may be diagnosed by combining the ultrasound and CTA findings.

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Pentalogy of Cantrell: Is Echocardiography Sufficient in the Neonatal Period?

Cited by 4 publications

References 6 publications

Pentalogy of Cantrell

Pentalogy of Cantrell

Experience in the Treatment of Pentalogy of Cantrell with Artificial Materials in a Single Clinical Center

Clinical and ultrasound findings of pentalogy of Cantrell in a newborn: A case report

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