Surgical palliation in patients with a single ventricle and dextrocardia

Poh, Chin; Xu, Mary; Galati, John C.; Iyengar, Ajay J.; Cheung, Michael; Konstantinov, Igor E.; d’Udekem, Yves

doi:10.1016/j.jtcvs.2013.10.077

Cited by 13 publications

(6 citation statements)

References 11 publications

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“…However, in contrast to published data, the mortality of patients undergoing TCPC was lower, but was not significantly different compared to children without DS.Poh et al. (2014) 17 AustraliaRoyal Children's Hospital, Melbourne1990–2008The medical data of 41 consecutive patients with single-ventricle physiology and dextrocardia in a single institution were investigated. In this cohort, 19 patients had heterotaxy syndrome.Twenty-five of the 41 patients had atrioventricular valve regurgitation (AVVR) on presentation (mild, 13; moderate, 9; severe, 3).The surgical outcomes of single ventricular palliation were less optimal in patients with dextrocardia.…”

Section: Resultsmentioning

confidence: 87%

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

Khoshhal

2019

Journal of Taibah University Medical Sciences

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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.

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Section: Resultsmentioning

confidence: 87%

“…Outcome of univentricular repair in patients with Down's syndrome (Furukawa et al., 2013) 16 Surgical palliation in patients with a single ventricle and dextrocardia (Poh et al., 2014) 17 High-risk single ventricle palliation in children with Down's syndrome: single institution experience (Ooi et al., 2015) 18 …”

Section: Methodsmentioning

confidence: 99%

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

Khoshhal

2019

Journal of Taibah University Medical Sciences

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“…However, according to a single institution study of patients with single-ventricle physiology and dextrocardia reported in 2014 by Chin L et al, only 56% of patients aged 15 years or younger with this condition survived, and the surgical outcome was poor. 10 Therefore, we consider that effective CPR for these patients is still important in emergency medicine. Recently, precision medicine has been emphasized.…”

Section: Discussionmentioning

confidence: 99%

“…5,6 However, the chest compression point recommended in current guidelines was the same for all cardiac arrest patients regardless of cardiac anomaly, including Fontan circulation patients with dextrocardia [7,8]. 7,8 In a recent study based on CT (Computed Tomography) analysis, researchers reported that the optimal compression point in patients with Fontan circulation undergoing CPR was approximately 5-25% of the lower sternum, which was lower than suggested in the current guidelines [9], however patients with dextrocardia, de ned as cardiac positioning with a rightward base-apex axis, [10,11] were not considered in the study. [9][10][11] Additionally, there have been no related studies on compression points in patients with dextrocardia.…”

Section: Introductionmentioning

confidence: 99%

Ideal Compression Point in Fontan Circulation Patients with Dextrocardia Undergoing Cardiopulmonary Resuscitation: An Analysis Based on Contrast-enhanced Chest Computed Tomography

Kim

Jung

Lee

et al. 2023

Preprint

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We aimed to find the ideal chest compression point undergoing cardiopulmonary resuscitation (CPR) in patients with a single ventricle with dextrocardia corrected by Fontan surgery. The most recent saved chest computed tomography images of all patients 8 years or older with a single ventricle who underwent Fontan surgery were retrospectively analysed. We defined the ideal chest compression point as the largest part of the compressed single ventricle. To find the ideal chest compression point, we measured the distance from the midline of the sternum to the point of the maximum sagittal area of the single ventricle as a deviation and calculated the area fraction of the compressed structures. Fifty-eight patients (male 67.2%) were analysed. The mean right deviation from the midline of the sternum as the ideal compression point was almost like the mean sternum width (32.85 ± 15.61 vs. 31.05 ± 6.75 mm). When chest compression was performed at the ideal point, the area fraction of the single ventricle statistically significant increased by 7%, wider than conventional compression (0.15 ± 0.10 vs. 0.22 ± 0.11, p < 0.05). When performing CPR on a patient with Fontan circulation with dextrocardia, right-sided chest compression is better than the conventional location.

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“…Mortality rates were 58% in isomerism and 27% in the non-isomerism groups. 18 In our study, we identified 12 out of 64 patients as Dextrocardia without isomerism (SSD). All SSD patients underwent single ventricle repair, and the mortality rate was 41% (5 out of 12).…”

Section: Discussionmentioning

confidence: 99%

Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery

et al. 2021

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Objective: Laterality anomalies are almost always associated with severe cardiac anomalies. Demographic properties, type of the procedures, associated anomalies, and early and mid-term prognosis of four types of laterality anomalies were analysed. Methods: A total of 64 consecutive patients with laterality anomalies were enrolled between July 2014 and July 2020. We grouped the patients as situs solitus dextrocardia (SSD) (n = 12; 18.7%); situs inversus (SI) (n = 16; 25%); right atrial isomerism (RAI) (n = 29; 45.3%); and left atrial isomerism (LAI) (n = 7; 10.9%). TAPVC was only present in the RAI group (31%). Incidence of mitral or tricuspid atresia was higher in the SSD group (25%). All the patients were followed up with a mean of 19.06 ± 17.6 (0.1–72) months. Results: Early postoperative mortality was 17 patients, among 107 procedures (15.8%). Twelve patients were in the neonatal period. All ten patients survived after isolated ductal stenting. Fourteen of the deaths were in the RAI group (48.3%). The 3-year survival rates were 85% in LAI, 78.7% in SI, 55.8% in SSD, and 38% in RAI groups. According to the multivariable Cox regression model, mechanical ventilation, kidney injury, RAI, and complex surgery in the neonatal period were independent risk factors for early mortality. Conclusion: Laterality anomalies are one of the most challenging patients who commonly had univentricular physiology. The most prevalent anomaly was RAI, and RAI had the worst outcome and survival. Ductal stent is an acceptable first intervention during the neonatal period in suitable patients. Complex procedures may carry a high risk of death in the neonatal period.

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Surgical palliation in patients with a single ventricle and dextrocardia

Abstract: Surgical outcomes of single-ventricle palliation seem poor in patients with dextrocardia. Aggressive management of congenital AVVR might improve the long-term prognosis.

Cited by 13 publications

References 11 publications

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

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

Ideal Compression Point in Fontan Circulation Patients with Dextrocardia Undergoing Cardiopulmonary Resuscitation: An Analysis Based on Contrast-enhanced Chest Computed Tomography

Analysis of outcomes in patients with abnormal laterality undergoing congenital heart surgery

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