Surgical palliation of univentricular heart disease in children with Down's syndrome: A systematic review

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Abstract

Objectives

No standard protocol is available for the management of children with Down's syndrome (DS) and a functional single ventricle. This review attempts to determine the outcomes of the single ventricular surgical palliation pathway in high-risk children with DS.

Methods

Several databases were searched using the following MeSH terms: ‘Congenital heart disease’, ‘Atrioventricular septal defect’, ‘Balanced AVSD’, ‘Unbalanced AVSD’, ‘Down's syndrome’, ‘Univentricular repair’, ‘bidirectional Glenn procedure’, and ‘Fontan procedure’. A structured algorithm was used for the selection of studies for an in-depth analysis.

Results

There was no universal agreement on the best surgical approach for unbalanced atrioventricular septal defect in DS. The majority of paediatric cardiac surgeons did not recommend the complete Fontan procedure; conversely, the use of a Glenn shunt (superior cavopulmonary connection) was preferred.

Conclusions

Careful assessment of the suitability for Fontan surgery, including the absence of elevated pulmonary vascular resistance, pulmonary arterial anatomy, and function of the dominant ventricle, is mandatory. A staged surgical procedure ending with complete Fontan repair provides acceptable medium-term results.

الملخص

أهداف البحث

لا يتوفر حاليا بروتوكول قياسي لعلاج الأطفال المصابين بمتلازمة داون والبطين الوظيفي الأحادي. يحاول هذا الاستعراض تحديد نتائج المسار الجراحي التلطيفي للبطين الأحادي لدى أطفال متلازمة داون ذوي الخطورة العالية.

طرق البحث

تم البحث في العديد من قواعد البيانات باستخدام شروط شبكة الموضوعات الطبية "مرض القلب الخلقي"، و"عيب الحاجز الأذيني البطيني"، و"عيب الحاجز الأذيني البطيني المتوازن "، و" عيب الحاجز الأذيني البطيني غير المتوازن"، و"متلازمة داون"، و"إصلاح البطين الأحادي"، و"طريقة جلين ثنائية الاتجاه" و" طريقة فونتان". واستخدمت خوارزمية منظمة لاختيار الدراسات لتحليل أعمق.

النتائج

لا يوجد اتفاق عالمي حول النهج الجراحي لمتلازمة داون مع عيب الحاجز الأذيني البطيني غير المتوازن. ولا ينصح معظم جراحي قلب الأطفال بالإجراء الكامل لطريقة فونتان، وبدلا عنها يفضلون تحويلة جلين (الوصل الأعلى للتجويف الرئوي).

الاستنتاجات

التقييم الدقيق لملائمة جراحة فونتان مثل عدم وجود ارتفاع في المقاومة الوعائية الرئوية، ووظيفة وتركيب الشريان الرئوي للبطين المهيمن يعتبر إلزاميا. والإجراء الجراحي على مراحل الذي ينتهي بإصلاح كامل بطريقة فونتان يوفر نتائج مقبولة متوسطة الأجل.

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Most cited references 35

Record: found
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Population-based study of congenital heart defects in Down syndrome.

S Freeman, L F Taft, K J Dooley … (1998)

Mental retardation and hypotonia are found in virtually all Down syndrome (DS) individuals, whereas congenital heart defects (CHDs) are only present in a subset of cases. Although there have been numerous reports of the frequency of CHDs in DS, few of the studies have had complete ascertainment of DS in a defined geographic area. The Atlanta Down Syndrome Project, a population-based study of infants born with trisomy 21, provides such a resource. In the first 6.5 years of the study, 243 trisomy 21 livebirths were identified in the five-county Atlanta area (birth prevalence: 9.6/10,000). Cardiac diagnoses were available on 227 (93%) of the cases and 89% of these evaluations were made by echocardiography, cardiac catheterization, surgery, or autopsy. Of the 227 DS infants, 44% had CHDs including 45% atrioventricular septal defect (with or without other CHDs), 35% ventricular septal defect (with or without other CHDs), 8% isolated secundum atrial septal defect, 7%, isolated persistent patent ductus arteriosus, 4% isolated tetralogy of Fallot, and 1% other. This report is unique in that it contains the largest number of trisomy 21 infants ascertained in a population-based study where modern techniques for diagnosing cardiac abnormalities predominate.

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Obstructive sleep apnea in children with Down syndrome.

Katharina von Bargen, S. Ward, Stephen Marcus … (1991)

Children with Down syndrome have many predisposing factors for the obstructive sleep apnea syndrome (OSAS), yet the type and severity of OSAS in this population has not been characterized. Fifty-three subjects with Down syndrome (mean age 7.4 +/- 1.2 [SE] years; range 2 weeks to 51 years) were studied. Chest wall movement, heart rate, electroculogram, end-tidal PO2 and PCO2, transcutaneous PO2 and PCO2, and arterial oxygen saturation were measured during a daytime nap polysomnogram. Sixteen of these children also underwent overnight polysomnography. Nap polysomnograms were abnormal in 77% of children; 45% had obstructive sleep apnea (OSA), 4% had central apnea, and 6% had mixed apneas; 66% had hypoventilation (end-tidal PCO2 greater than 45 mm Hg) and 32% desaturation (arterial oxygen saturation less than 90%). Overnight studies were abnormal in 100% of children, with OSA in 63%, hypoventilation in 81%, and desaturation in 56%. Nap studies significantly underestimated the presence of abnormalities when compared to overnight polysomnograms. Seventeen (32%) of the children were referred for testing because OSAS was clinically suspected, but there was no clinical suspicion of OSAS in 36 (68%) children. Neither age, obesity, nor the presence of congenital heart disease affected the incidence of OSA, desaturation, or hypoventilation. Polysomnograms improved in all 8 children who underwent tonsillectomy and adenoidectomy, but they normalized in only 3. It is concluded that children with Down syndrome frequently in have OSAS, with OSA, hypoxemia, and hypoventilation. Obstructive sleep apnea syndrome is seen frequently in those children in whom it is not clinically suspected. It is speculated that OSAS may contribute to the unexplained pulmonary hypertension seen in children with Down syndrome.

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Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

J. Lock, Carlos A. Castaneda, J Mayer … (1997)

The purpose of this study was to review a large, evolving, single-center experience with the Fontan operation and to determine risk factors influencing early and late outcome. The first 500 patients undergoing modifications of the Fontan operation at our institution were identified. Perioperative variables were recorded and a cross-sectional review of survivors was undertaken. The incidence of early failure decreased from 27.1% in the first quartile of the experience to 7.5% in the last quartile. In a multivariate model, the following variables were associated with an increased probability of early failure: a mean preoperative pulmonary artery pressure of 19 mm Hg or more (p < 0.001), younger age at operation (p = 0.001), heterotaxy syndrome (p = 0.03), a right-sided tricuspid valve as the only systemic atrioventricular valve (p = 0.001), pulmonary artery distortion (p = 0.04), an atriopulmonary connection originating at the right atrial body or appendage (p = 0.001), the absence of a baffle fenestration (p = 0.002), and longer cardiopulmonary bypass time (p = 0.001). An increased probability of late failure was associated with the presence of a pacemaker before the Fontan operation (p < 0.001). A morphologically left ventricle with normally related great arteries or a single right ventricle (excluding heterotaxy syndrome and hypoplastic left heart syndrome) were associated with a decreased probability of late failure (p = 0.003). These analyses indicate that early failure has declined over the study period and that this decline is related in part to procedural modifications. A continuing late hazard phase is associated with few patient-related variables and does not appear related to procedural variables.

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Author and article information

Contributors

Saad Q. Khoshhal

Journal

Journal ID (nlm-ta): J Taibah Univ Med Sci

Journal ID (iso-abbrev): J Taibah Univ Med Sci

Title: Journal of Taibah University Medical Sciences

Publisher: Taibah University

ISSN (Electronic): 1658-3612

Publication date PMC-release: 01 December 2018

Publication date Collection: February 2019

Publication date (Electronic): 01 December 2018

Volume: 14

Issue: 1

Pages: 1-7

Affiliations

[1]Taibah University, Medical College – Paediatric Department, Almadinah Almunawwarah, KSA

Author notes

[∗ ]Corresponding address: Taibah University, Medical College – Paediatric Department, Almadinah Almunawwarah, KSA drsaad68@ 123456hotmail.com

Article

Publisher ID: S1658-3612(18)30113-6

DOI: 10.1016/j.jtumed.2018.10.006

PMC ID: 6694996

SO-VID: b82e89c4-2122-4896-b945-308ae0b91fa6

License:

This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

History

Date received : 11 July 2018

Date revision received : 27 October 2018

Date accepted : 28 October 2018

Surgical palliation of univentricular heart disease in children with Down's syndrome: A systematic review

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Abstract

Objectives

Methods

Results

Conclusions

الملخص

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Most cited references 35

Population-based study of congenital heart defects in Down syndrome.

Obstructive sleep apnea in children with Down syndrome.

Fontan operation in five hundred consecutive patients: factors influencing early and late outcome.

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