Survival into adulthood of patients with unoperated single ventricle

doi:10.1016/S0002-9149(97)89356-3

The American Journal of Cardiology

Volume 77, Issue 7, 1 March 1996, Pages 542-544

https://doi.org/10.1016/S0002-9149(97)89356-3 Get rights and content

Abstract

Adult patients with double-inlet left ventricle and perfectly balanced circulation may survive into the sixth decade with good functional capacity and preserved ventricular function. This should be considered before such patients are referred for a Fontan repair.

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Cited by (60)

Univentricular hearts not undergoing Fontan: the type of palliation matters
2024, Revista Espanola de Cardiologia
La información sobre pacientes con fisiología univentricular (FU) y flujo pulmonar restrictivo no sometidos a cirugía de Fontan es escasa. El objetivo de este estudio es comparar la supervivencia y los eventos cardiovasculares en estos pacientes según el tipo de paliación.
Los datos de pacientes con FU se obtuvieron de las bases de datos de 7 centros con unidades de cardiopatías congénitas del adulto. Se excluyó a los pacientes que completaron la circulación de Fontan o desarrollaron un síndrome de Eisenmenger. Se crearon 3 grupos según la fuente de flujo pulmonar: G1, flujo anterógrado pulmonar restrictivo; G2, shunt cavopulmonar, y G3, shunt aortopulmonar ± shunt cavopulmonar. El objetivo principal fue la muerte.
Se identificó a 120 pacientes. La media de edad en la primera visita fue 32,2 años. El seguimiento medio fue de 7,1 años. Se asignó a 55 pacientes (45,8%) al G1, 30 (25%) al G2 y 35 (29,2%) al G3. Los pacientes del G3 tenían peores función renal, clase funcional y fracción de eyección en la primera visita y mostraron una disminución más marcada de la fracción de eyección durante el seguimiento, especialmente en comparación con el G1. Veinticuatro pacientes (20%) fallecieron, 38 (31,7%) ingresaron por insuficiencia cardiaca y 21 (17,5%) presentaron aleteo/fibrilación auricular durante el seguimiento. Estos eventos fueron más frecuentes en el G3 y al compararlos con los del G1 se encontraron diferencias significativas en muerte (HR = 2,9; IC95%, 1,14-7,37; p = 0,026) y aleteo/fibrilación auricular (HR = 2,9; IC95%, 1,11-7,68; p = 0,037).
El tipo de paliación de los pacientes con FU y flujo pulmonar restrictivo no sometidos a cirugía de Fontan identifica distintos perfiles. Los pacientes paliados con derivaciones aortopulmonares presentan un peor pronóstico, con más morbilidad y mortalidad.
There is scarce information on patients with single ventricle physiology (SVP) and restricted pulmonary flow not undergoing Fontan circulation. This study aimed to compare survival and cardiovascular events in these patients according to the type of palliation.
SVP patient data were obtained from the databases of the adult congenital heart disease units of 7 centers. Patients completing Fontan circulation or developing Eisenmenger syndrome were excluded. Three groups were created according to the source of pulmonary flow: G1 (restrictive pulmonary forward flow), G2 (cavopulmonary shunt), and G3 (aortopulmonary shunts ± cavopulmonary shunt). The primary endpoint was death.
We identified 120 patients. Mean age at the first visit was 32.2 years. Mean follow-up was 7.1 years. Fifty-five patients (45.8%) were assigned to G1, 30 (25%) to G2, and 35 (29.2%) to G3. Patients in G3 had worse renal function, functional class, and ejection fraction at the first visit and a more marked ejection fraction decline during follow-up, especially when compared with G1. Twenty-four patients (20%) died, 38 (31.7%) were admitted for heart failure, and 21 (17.5%) had atrial flutter/fibrillation during follow-up. These events were more frequent in G3 and significant differences were found compared with G1 in terms of death (HR, 2.9; 95%CI, 1.14-7.37; P = .026) and atrial flutter/fibrillation (HR, 2.9; 95%CI, 1.11-7.68; P = .037).
The type of palliation in patients with SVP and restricted pulmonary flow not undergoing Fontan palliation identifies distinct profiles. Patients palliated with aortopulmonary shunts have an overall worse prognosis with higher morbidity and mortality.
The “Forgotten Ones”: The Natural and Unnatural History of Univentricular Physiology Without Fontan Palliation
2022, Canadian Journal of Cardiology
Long-term Outcomes of Adults With Single Ventricle Physiology Not Undergoing Fontan Repair: A Multicentre Experience
2022, Canadian Journal of Cardiology
Citation Excerpt :
Long-term survival in patients with SVP who had not undergone Fontan palliation is unusual. However, those cases with a good balance between systemic and pulmonary blood flows5-8,13 or some patients with Eisenmenger syndrome9,14 may reach adulthood with a fairly good quality of life. Gatzoulis et al.5 published an interesting retrospective study including 50 patients with cavopulmonary or aortopulmonary shunts as definitive palliation.
To describe long-term survival and cardiovascular events in adult patients with single ventricle physiology (SVP) without Fontan palliation, focusing on predictors of mortality and comparing groups according to their cardiovascular physiology.
Multicentre observational and retrospective study including adult patients with SVP without Fontan palliation since their first adult clinic visit. The cohort was subdivided into 3 groups: Eisenmenger, restricted pulmonary flow, and aortopulmonary shunt. Death was considered as the main end point. Other clinical outcomes occurring during follow-up were considered as secondary end points.
A total of 146 patients, mean age 32.5 ± 11.1 years, were analysed. Over a mean follow-up of 7.3 ± 4.1 years, 33 patients (22.6%) died. Survival was 86% and 74% at 5 and 10 years, respectively. Right ventricular morphology was not associated with higher mortality. Four variables at baseline were related to a higher mortality: at least moderate atrioventricular valve regurgitation, platelet count < 150 × 10³/mm³, GFR < 60 mL/min/1.73 m², and QRS > 120 ms). A total of 34.2% of patients were admitted to the hospital due to heart failure, and 7.5% received a heart transplant. Other cardiovascular outcomes were also frequent: atrial arrhythmias in 19.2%, stroke in 15.1%, and pacemaker/implantable cardioverter-defibrillator in 6.2%/2.7%.
Adult patients with SVP who had not undergone Fontan exhibit a high mortality rate and frequent major cardiovascular events. At least moderate atrioventricular valve regurgitation, thrombocytopenia, renal dysfunction, and QRS duration > 120 ms at baseline visit allow identification of a cohort of patients at higher risk of mortality.
Décrire la survie à long terme et les événements cardiovasculaires chez les patients adultes présentant une physiologie à ventricule unique (PVU) sans intervention de Fontan, en se concentrant sur les prédicteurs de mortalité et en comparant les groupes en fonction de leur physiologie cardiovasculaire.
Étude observationnelle et rétrospective, multicentrique, incluant des patients adultes atteints de PVU sans intervention de Fontan depuis leur première visite en clinique adulte. La cohorte a été subdivisée en trois groupes : syndrome d'Eisenmenger, flux pulmonaire restreint ou fenêtre aorto-pulmonaire. Le décès a été considéré comme le critère principal d'évaluation. Les autres observations cliniques survenues au cours du suivi ont été considérées comme des critères secondaires.
Un total de 146 patients, d'un âge moyen de 32,5 ± 11,1 ans, a été considéré pour l’analyse. Sur un suivi moyen de 7,3 ± 4,1 ans, 33 patients (22,6 %) sont décédés. La survie était de 86 % et 74 % à 5 et 10 ans, respectivement. La morphologie du ventricule droit n'était pas associée à une mortalité plus élevée. Quatre variables initiales étaient liées à une mortalité plus élevée : régurgitation au moins modérée de la valve auriculo-ventriculaire, numération plaquettaire < 150 × 10³/mm³, DFG < 60 ml/min/1,73 m² et QRS > 120 ms). Au total, 34,2 % des patients ont été admis à l'hôpital en raison d'une insuffisance cardiaque, et 7,5 % ont reçu une transplantation cardiaque. D'autres conséquences cardiovasculaires étaient également fréquentes : arythmies auriculaires dans 19,2 % des cas, accident vasculaire cérébral dans 15,1 % des cas et stimulateur cardiaque/ défibrillateur cardioverteur implantable dans 6,2 % / 2,7 % des cas.
Les patients adultes atteints de PVU qui n'ont pas subi d'intervention de Fontan présentent un taux de mortalité élevé et des événements cardiovasculaires majeurs fréquents. Une régurgitation au moins modérée de la valve auriculo-ventriculaire, une thrombocytopénie, une dysfonction rénale et une durée du QRS > 120 ms lors de la visite initiale permettent de distinguer une cohorte de patients présentant un risque de mortalité plus élevé.
Fate of Patients With Single Ventricles Who Do Not Undergo the Fontan Procedure
2022, Annals of Thoracic Surgery
Citation Excerpt :
Patients born with worse subtypes had 50% mortality within 4 years.9 Two subsequent studies from the Mayo Clinic, including a review of all reported cases of unoperated adult survivors with single ventricles in the literature, similarly described that select patients with DILV, pulmonary stenosis, and a “balanced” physiology may survive with good functional status to the eighth decade of life.10,11 Over the last 2 decades, the introduction of treatment with pulmonary vasodilators seemed to have improved the survival of patients with Eisenmenger syndrome.
The Fontan procedure, the last of a series of palliative operations for patients born with single ventricles, is associated with a significant late burden of complications. There are other strategies for patients who are suboptimal candidates for Fontan completion; however, the long-term outcomes of these different surgical options have not been clearly elucidated. We performed a systematic literature review to establish the current role of other treatment approaches besides the Fontan procedure.
The MEDLINE and Embase databases were systematically searched for articles describing the long-term outcomes of patients with single ventricles who have not received the Fontan procedure.
A total of 36 articles met all inclusion criteria. There is a scarcity of contemporary data on the non-Fontan cohort. Historical studies provided a significant contribution.
Long-term survival of unoperated patients with single ventricles is possible under the rare conditions of having balanced hemodynamics. As many as half of patients may survive on only a systemic-to-pulmonary artery shunt or bidirectional cavopulmonary shunt for more than 20 years with reasonable functional status. In patients with a failing single ventricle, the bidirectional cavopulmonary shunt is an excellent bridge to heart transplantation and may provide better posttransplant survival than patients with a Fontan circulation. Currently, the Fontan procedure continues to be the best definitive palliation for patients born with single ventricle lesions. However, for those with borderline indications, other strategies should be carefully considered.
Abdominal Imaging Surveillance in Adult Patients After Fontan Procedure: Risk of Chronic Liver Disease and Hepatocellular Carcinoma
2018, Current Problems in Diagnostic Radiology
To assess the prevalence of chronic liver disease (CLD) and hepatocellular carcinoma (HCC) in adult patients who had surveillance imaging after Fontan procedure.
Institutional review board–approved, Health Insurance Portability and Accountability Act–compliant retrospective study evaluated electronic medical records including radiology reports and clinical notes for adult patients after Fontan procedure between January 1993 and January 2016. Abdominal ultrasound, computed tomography, and magnetic resonance imaging reports were reviewed for changes of CLD and HCC. Existence of concomitant viral hepatitis was also recorded.
A total of 145 patients (male: 78 and female: 67) had surveillance imaging after Fontan procedure. In all, 78% (113/145) had ongoing imaging surveillance (median follow-up 3.05 years, IQR: 0.75-5.3 years); 19% (21/113) had an initial normal study and remained normal throughout follow-up; 19% (21/113) had an initial normal study with subsequent imaging reporting changes of CLD; and 62% (71/113) had existing changes of CLD on initial study. HCC was identified in 5 patients (median 22 years post-Fontan, IQR: 10-29 years), 4 of which had a normal initial study. Only 1 patient with HCC had concomitant viral hepatitis C infection.
Radiologists should be aware that CLD is exceedingly common in post-Fontan cardiac physiology, and surveillance imaging is warranted given the risk of HCC.
Successful pregnancy and delivery in patients with uncorrected single ventricle: Three new cases and literature review
2015, International Journal of Cardiology
Citation Excerpt :
In general, women with NYHA class I–II have a relatively favorable prognosis [26]. In addition, pulmonary hypertension was associated with poor prognosis and thought to be more serious than cyanosis in pregnancy [6,23–27]. Pulmonary hypertension can lead to polycythemia, a decrease in arterial oxygen saturation, and an increase in right-to-left shunting.
Due to high risks of both maternal and fetal complications, pregnancy is not encouraged for women with uncorrected univentricular heart (UVH). Here, we report three cases of successful pregnancy and delivery in patients with uncorrected UVH. A literature review has been performed. It appears that maternal and neonatal risks are mainly associated with higher NYHA heart failure class, pulmonary hypertension, and history of congestive heart failure. In the absence of these risk factors, successful pregnancy still can be achieved with mild complications. Care by a multidisciplinary team during delivery is necessary to for a good prognosis.

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¹: Dr. Warnes' address is: Mayo Clinic, 200 First Street SW, Rochester, Minnesota 55905.

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Brief reportSurvival into adulthood of patients with unoperated single ventricle

Abstract

Am Heart J

Am J Cardiol

Am J Cardiol

J Am Coll Cardiol

Am J Cardiol

Brief report
Survival into adulthood of patients with unoperated single ventricle