Heart Transplant for Failing Fontan with Situs Inversus and Aortic Aneurysm: Utilization of Lateral Tunnel Fontan Pathway for Systemic Venous Reconstruction

Babies born with a single functioning heart ventricle instead of two require a series of surgeries during the first few years of life to redirect their blood flow, resulting in a Fontan circulation. Patients with Fontan circulations have excellent early survival; however, over time, their circulations begin to fail, ultimately resulting in their death. Currently, the only treatment for failing Fontan circulation is a heart transplant; however, many Fontan patients do not survive long enough to receive a donor heart. One of the reasons for this is a lack of understanding of the Fontan failure cascade. Often patients are identified as failing when they do not have enough time left to receive a heart transplant. The objective of this problem is to develop mathematical models of healthy and failing Fontan circulations to i) improve our understanding of Fontan failure from a hemodynamic perspective, and ii) identify physiologically-relevant ranges of parameters.

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Abdominal Aortic Aneurysm in Situs Inversus Totalis

Annals of Vascular Surgery ◽

10.1007/s10016-006-9015-1 ◽

2006 ◽

Vol 20 (2) ◽

pp. 267-270 ◽

Cited By ~ 7

Author(s):

Takayoshi Kato ◽

Hisato Takagi ◽

Seishiro Sekino ◽

Hideaki Manabe ◽

Yukihiro Matsuno ◽

...

Keyword(s):

Abdominal Aortic Aneurysm ◽

Aortic Aneurysm ◽

Situs Inversus ◽

Situs Inversus Totalis ◽

Abdominal Aortic

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Rupture of an abdominal aortic aneurysm in a patient with a situs inversus totalis: Report of a case

Surgery Today ◽

10.1007/s00595-007-3616-3 ◽

2008 ◽

Vol 38 (3) ◽

pp. 249-252 ◽

Cited By ~ 8

Author(s):

Naoyuki Kimura ◽

Koji Kawahito ◽

Satoshi Ito

Keyword(s):

Abdominal Aortic Aneurysm ◽

Aortic Aneurysm ◽

Situs Inversus ◽

Situs Inversus Totalis ◽

Abdominal Aortic

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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

The Journal of Heart and Lung Transplantation ◽

10.1016/j.healun.2006.12.002 ◽

2007 ◽

Vol 26 (3) ◽

pp. 290-292 ◽

Cited By ~ 4

Author(s):

A. Beiras-Fernandez ◽

S.H. Daebritz ◽

I. Kaczmarek ◽

R. Kozlik-Feldmann ◽

A.R. Tiete ◽

...

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Heart Transplantation ◽

Situs Inversus ◽

Congenital Heart ◽

Fontan Circulation ◽

Complex Congenital Heart Disease ◽

Venous Reconstruction

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Multistage Endovascular Management of a Thoracoabdominal Aortic Aneurysm in a Post–Heart Transplant Patient

Vascular and Endovascular Surgery ◽

10.1177/15385744211056142 ◽

2021 ◽

pp. 153857442110561

Author(s):

Maciej Malinowski ◽

Jakub Młodzik ◽

Grzegorz Jodłowski ◽

Artur Borkowski ◽

Jan Skóra ◽

...

Keyword(s):

Aortic Aneurysm ◽

Ejection Fraction ◽

Endovascular Treatment ◽

Heart Transplant ◽

Transplant Patient ◽

Thoracoabdominal Aortic Aneurysm ◽

Clinical Situation ◽

Heart Transplant Patient ◽

Endovascular Management ◽

Thoracoabdominal Aorta

The development of aneurysms of thoracoabdominal aorta (TAAA) in a post-transplant patient is a rare clinical situation and requires special attention. Endovascular treatment is the most suitable option for these patients due to numerous comorbidities. Particular emphasis should be placed on the ejection fraction as one of the main criteria for qualifying for surgery. The treatment itself remains a major challenge relating to anatomical constrains; however, it is possible in select patients in experienced centers.

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Abstract 18929: Pacing in the Adult Fontan Patient: Risky Business?

Circulation ◽

10.1161/circ.130.suppl_2.18929 ◽

2014 ◽

Vol 130 (suppl_2) ◽

Author(s):

Matthew J Lewis ◽

Nalini Colaco ◽

Jonathan Ginns ◽

Marlon Rosenbaum

Keyword(s):

Functional Status ◽

Primary Endpoint ◽

Heart Transplant ◽

Fold Increase ◽

Fontan Patient ◽

Risk Of Death ◽

Lateral Tunnel ◽

Increased Risk ◽

Fontan Patients

Introduction: Chronic ventricular pacing can induce a cardiomyopathy in patients with a biventricular heart; however, the effect of chronic pacing in adult patients with a Fontan has not been well characterized. We hypothesized that paced adult Fontan patients would be at higher risk for death or heart transplant. Methods: We performed a retrospective, cohort of study of all adult Fontan patients at the Schneeweiss Adult Congenital Heart Center seen between 1/1997 and 5/2014. Two Cohorts were defined based on whether a patient did or did not have a permanent pacemaker. Demographic and clinical characteristics were collected via chart review. The primary endpoint was a composite of death or heart transplant. Results: Of the 98 adult Fontan patients followed (mean age at last follow-up 32± 8 years), 30 (31%) had a pacemaker. Pacemaker specific data was available on 25 of the 30 (83%) paced patients. Of those, 88% were paced >50% of the time. Patient diagnoses included double inlet left ventricle in 33 (34%), tricuspid atresia in 26 (27%), hypoplastic left heart in 9 (9%), heterotaxy in 8 (8%), and 22 (22%) with other diagnoses. Fifty-two patients (53%) had a classic RA-PA Fontan and 46 (47%) had a lateral tunnel or extracardiac Fontan. Over the study period, 16 patients met the primary endpoint and 12 (75%) were paced. Paced patients were significantly more likely to have worse functional status (p<0.001), be on diuretics (p<0.001), and have a higher mean creatinine (P=0.025), mean total bilirubin (p=0.025), and mean Fontan pressure (p<0.001). Pacing was associated with >4-fold increase in the rate of death or heart transplant (p=0.009) in a multivariate cox-proportional hazard model that included Fontan type, age at Fontan completion, age at follow-up, and pacing status. Discussion: In our cohort of 98 adult Fontan patients, paced patients were more likely to have worse functional status, require diuretics and had a >4-fold increased risk of death or heart transplant. These results suggest that chronic pacing may be detrimental in this population.

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Orthotopic heart transplant following implantation of a systemic ventricular assist device in a patient with congenitally corrected transposition of the great arteries and dextrocardia situs inversus totalis

The VAD Journal ◽

10.14434/vad.v2i0.27962 ◽

2016 ◽

Cited By ~ 1

Author(s):

Bree Ann C. Young ◽

Zachariah B. Nealy ◽

Michael F. McGrath ◽

John M. Herre

Keyword(s):

Heart Failure ◽

Heart Transplant ◽

Ventricular Assist Device ◽

Situs Inversus ◽

Advanced Heart Failure ◽

Assist Device ◽

Orthotopic Heart Transplant ◽

Ventricular Assist ◽

Congenitally Corrected Transposition ◽

Corrected Transposition

Congenitally corrected transposition of the great arteries (CCTGA) is a rare congenital heart disease that commonly results in congestive heart failure due to exposure of the anatomic right ventricle to systemic pressures. Additional anatomic abnormalities, such as situs inversus, in which the viscera are mirror images of normal anatomy, or dextrocardia in which the heart apex is located on the patient’s right, once were contraindications to transplant in the treatment of advanced heart failure. However, recent advancements now allow for the management of heart failure through mechanical circulatory support and subsequent transplant. This case presents a 52 year-old man with advanced heart failure secondary to CCTGA and dextrocardia situs inversalis totalis that was managed with a ventricular assist device (VAD) after maximal medical treatment, and subsequently received an orthotopic heart transplant. This case will also review CCTGA in relation to heart failure and the surgical challenges faced in terms of ventricular assist device implantation and orthotopic heart transplant.

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Pararenal aortic aneurysm in situs inversus totalis: open repair with right retroperitoneal approach

Journal of Surgical Case Reports ◽

10.1093/jscr/rjaa197 ◽

2020 ◽

Vol 2020 (7) ◽

Author(s):

Emanuele Gatta ◽

Sara Schiavon ◽

Gabriele Pagliariccio ◽

Perla Persechini ◽

Luciano Carbonari

Keyword(s):

Abdominal Aortic Aneurysm ◽

Aortic Aneurysm ◽

Situs Inversus ◽

Open Repair ◽

Situs Inversus Totalis ◽

Mirror Image ◽

Operative Risk ◽

Retroperitoneal Approach ◽

Abdominal Aortic ◽

Image Reversal

Abstract Situs inversus totalis (SIT) refers to a mirror-image reversal of the internal organ position. The coexistence of abdominal aortic aneurysm and SIT is extremely rare; very short series have been reported; therefore its prevalence has never been estimated. The presence of anatomical anomalies could increase operative risk. The authors describe a case of successfully repaired pararenal abdominal aortic aneurysm with a right retroperitoneal approach in situs inversus totalis.

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