Challenging Venous Reconstruction and Heart Transplantation in a Patient With Viscero-atrial Situs Inversus and Complex Congenital Heart Disease With Fontan Circulation

Beiras-Fernandez, A.; Daebritz, Sabine; Kaczmarek, Ingo; Kozlik‐Feldmann, Rainer; Tiete, A.; Reichart, Bruno

doi:10.1016/j.healun.2006.12.002

Cited by 6 publications

(2 citation statements)

References 5 publications

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“…The primary strength of our analysis is the ability to identify and analyze patients with heterotaxy in a large, multicenter cohort, thereby providing novel insights into post-HT survival as well as peri-operative complications, procedures, and resource utilization for the HT hospitalization via analysis of ICD codes captured by PHIS. We hypothesized that patients with heterotaxy have worse perioperative outcomes due to increased surgical complexity related to anomalies of cardiac position and venous return [4,[23][24][25][26]. Indeed, we have found evidence of this.…”

Section: Discussionmentioning

confidence: 86%

Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements

Duong

Godown

Soslow

et al. 2019

The Journal of Thoracic and Cardiovascular Surgery

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Objectives: Heart transplantation (HT) in children with heterotaxy may be affected by anomalous cardiac position, venous return, and splenic function. Outcomes are not well described. We compared post-HT outcomes, including survival and resource utilization, among recipients with congenital heart disease (CHD) in the presence and absence of heterotaxy. Methods: Using linked Pediatric Health Information System and Scientific Registry of Transplant Recipients data (2001-2016), we identified 177 HT recipients with heterotaxy. We compared post-HT outcomes to 1,202 non-heterotaxy recipients with CHD in multivariable regression models. Length of stay (LOS) and cost from HT to discharge were also compared. Results: Heterotaxy HT recipients were older (median age 5.1 vs. 1.6 years, p<0.001) and more often Black, Asian, Hispanic, or "other" non-Caucasian (54 vs. 32%, p<0.001). Heterotaxy was independently associated with increased mortality (HR 1.61, [95% CI 1.20-2.15], p=0.001), even among 6-month survivors (HR 2.00 [1.17-3.43], p=0.012). Heterotaxy recipients more commonly required dialysis (OR 2.64 [1.52-4.56], p=0.001) and cardiac reoperation (OR 2.36 [1.29-3.41], p=0.003) prior to discharge. They had longer ischemic times (19 additional minutes [10.4-27.6], p<0.001, adjusted for organ procurement distance), ICU LOS (16 vs. 13 days, p=0.012), and hospital LOS (median 26 vs. 23 days, p=0.005). Post-HT hospitalization costs were also greater

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Section: Discussionmentioning

confidence: 86%

Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements

Duong

Godown

Soslow

et al. 2019

The Journal of Thoracic and Cardiovascular Surgery

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“…As a result, there has been limited publication about heart transplants that involve recipients with dextrocardia situs inversus and CHD and donors with normal hearts in situs solitus. The reports we found mainly discussed the structural disorders of vascular roots such as mirrored vena cava and transposition of great arteries, or palliative procedure like Fontan [5]. We had yet to encounter any report about supracardiac type total anomalous pulmonary venous connection, which means the recipient does not have a normal heart atrium required for the most important anastomosis in transplant.…”

Section: Discussionmentioning

confidence: 99%

Heart Transplant for Dextrocardia Situs Inversus with Very Complex Congenital Lesion: a Challenge of Cardiac Surgery

Uoc¹

2015

Int J Clin Cardiol

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Background: Viet Duc Hospital has carried out 9 out of 11 heart transplants in Vietnam since 2010. The vast majority of cases is a heart transplant at the same location i.e. the cardiac stem of the recipient is of the same anatomy with that of the donor. In congenital heart disease (CHD), if the cardiac stem of the recipient is of a disordered structure, it will pose a major challenge for the heart transplant itself as well as the recovery following transplantation. The team at Viet Duc hospital carried out a successful transplant in a particularly complicated case of CHD in May 2014. This report aims to give an overview of this unique procedure and its results. Method: clinical case report.Results: 27 year-old female patient had complex CHD that involved dextrocardia situs inversus, double outlet right ventricle type TGA, pulmonary artery stenosis, total atrio-ventricular canal, and total anomalous pulmonary venous connection type supra-cardia. She was experiencing end-stage heart failure and severely cachexia with risk of death within few months. The transplanted heart was from a multi-organ donor; male, 26 years old, normal heart structure. Although the difference in biological parameters between donor and recipient were with in acceptable limits, most of the steps in an otherwise normal transplant proved to be highly challenging. For instance, the team had to create a new cardiac cavity on left chest and the "left" atrium chamber at a suitable location, repair and transfer the pulmonary artery to the posterior-left position, and transfer the superior-inferior vena cava to the right location. The transplant lasted 9 hours with 170 minutes of aortic cross-clamp time. Recovery from surgery was highly chalenging with risks of multi-organ failure and ventilator-associated lung injury. Intra-aortic balloon pump was used for 8 days, continuous renal replacement therapy for 5 weeks, tracheostomy and mechanical ventilation for 10 weeks. Total time in hospital post-surgery was 3.5 months. Good improvement was observed at one-year follow-up. Conclusion:This is a very rare case of heart transplant which was further complicated by pre-existing dextrocardia. It presented highly uncommon challenges that called for inventive techniques. The team's experience in managing complex CHD and cardiac transplant was the most important factor that contributed to the success of the procedure.

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Abnormalities of Situs

Marino¹,

Versacci²,

Guccione³

et al. 2012

Pediatric Cardiovascular Medicine

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Challenging Venous Reconstruction and Heart Transplantation in a Patient With Viscero-atrial Situs Inversus and Complex Congenital Heart Disease With Fontan Circulation

Cited by 6 publications

References 5 publications

Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements

Increased mortality, morbidities, and costs after heart transplantation in heterotaxy syndrome and other complex situs arrangements

Heart Transplant for Dextrocardia Situs Inversus with Very Complex Congenital Lesion: a Challenge of Cardiac Surgery

Abnormalities of Situs

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