Survival of Double Inlet Left Ventricle Patients Without Fontan Circulation

NCT03667144 | Unknown DMC

• 1 other identifier: NL59516.042.17
• Study Type: observational
• Target: 15
• Locations: 0 countries
• Sites: N/A

Brief Summary

Patients with univentricular hearts are currently palliated with the Fontan procedures. This results in an unphysiologic circulation with poor long-term survival. On the other hand there is a small, selected subgroup of patients with univentricular hearts of the double inlet left ventricle (DILV) type that survives up to old age without ever having undergone any Fontan procedures. Considering the relatively bleak data on long-term survival of DILV patients palliated with the Fontan procedure, it seems highly relevant to investigate and determine factors that allow a selection of unoperated patients to live to a comparatively high age without apparent major complaints. This might have an impact on how (and if, at all) certain univentricular patients are selected for operation in the future. For some, a different surgical procedure, or even therapeutic nihilism might be a more viable alternative. The investigators hypothesise that favourable intracardiac streaming plays a role in making these patients viable: certain intracardiac anatomical characteristics allow for favourable flow patterns. The primary objective of this study is to describe the intracardiac anatomy and hemodynamics of DILV patients without Fontan circulation using 4D MRI. Furthermore, the functional status and quality of life of these patients will be assessed.

Conditions

Double Inlet Left Ventricle

Keywords

Double inlet left ventricle without Fontan circulation

Outcome Measures

Primary Outcomes (1)

• Angle between atrioventricular and ventriculoarterial valves

  This is primarily an explorative study aiming to describe the anatomy of unpalliated DILV hearts. With 4D MRI, the hearts will be grouped according to the angular relationship of the atrioventricular to the ventriculo-arterial valves. This angle can be divided into three categories: 1) parallel (i.e. 0°), 2) perpendicular (i.e. 90°) or 3) an in-between variant. Accordingly, flow patterns, ranging from linear to turbulent will be measured and correlated to the anatomical variants.

  1 day

Secondary Outcomes (18)

• New York Heart Association Functional Classification

  1 day

• Peak VO2

  1 day

• Peripheral oxygen saturation

  1 day

• Scores for Quality of life Adults

  1 day

• Scores for Quality of life

  1 day

Other Outcomes (3)

• Demographics

  1 day

• Highest Level of Education

  1 day

• Current Medication use

  1 day

Eligibility Criteria

• Age: 12 Years+
• Sex: all
• Healthy Volunteers: No
• Age Groups: Child (0-17), Adult (18-64), Older Adult (65+)
• Sampling Method: Non-Probability Sample

Study Population

Included will be patients over the age of 12 with the diagnosis double inlet left ventricle, which have not undergone the Fontan trajectory. For the 4D MRI, patients with an MRI non-compatible pacemaker in situ will be excluded.

You may qualify if:

• Diagnosis double inlet left ventricle, established by echocardiograph or MRI
• Not undergone Fontan trajectory in the past
• Be of the age of 12 or older
• Written informed consent

You may not qualify if:

• previous (partial) cavopulmonary connection
• The following subjects will be excluded from the MRI part of the study, but will still be asked to participate in all the other parts:
• MRI non-compatible pacemaker in situ

Sponsors & Collaborators

• University of Groningen: lead

Related Publications (8)

• Frescura C, Thiene G. The new concept of univentricular heart. Front Pediatr. 2014 Jul 7;2:62. doi: 10.3389/fped.2014.00062. eCollection 2014.

  PMID: 25072035 BACKGROUND

• Cook AC, Anderson RH. The anatomy of hearts with double inlet ventricle. Cardiol Young. 2006 Feb;16 Suppl 1:22-6. doi: 10.1017/S1047951105002283. No abstract available.

  PMID: 16401359 BACKGROUND

• Franklin RC, Spiegelhalter DJ, Anderson RH, Macartney FJ, Rossi Filho RI, Douglas JM, Rigby ML, Deanfield JE. Double-inlet ventricle presenting in infancy. I. Survival without definitive repair. J Thorac Cardiovasc Surg. 1991 May;101(5):767-76.

  PMID: 2023434 BACKGROUND

• Gersony WM. Fontan operation after 3 decades: what we have learned. Circulation. 2008 Jan 1;117(1):13-5. doi: 10.1161/CIRCULATIONAHA.107.748566. No abstract available.

  PMID: 18172049 BACKGROUND

• Poterucha JT, Anavekar NS, Egbe AC, Julsrud PR, Connolly HM, Ammash NM, Warnes CA. Survival and outcomes of patients with unoperated single ventricle. Heart. 2016 Feb;102(3):216-22. doi: 10.1136/heartjnl-2015-308440. Epub 2015 Dec 23.

  PMID: 26701967 BACKGROUND

• Macartney FJ, Partridge JB, Scott O, Deverall PB. Common or single ventricle. An angiocardiographic and hemodynamic study of 42 patients. Circulation. 1976 Mar;53(3):543-54. doi: 10.1161/01.cir.53.3.543.

  PMID: 1248087 BACKGROUND

• Wolff D, van Melle JP, Bartelds B, Ridderbos FS, Eshuis G, van Stratum EBHJ, Recinos SJ, Willemse BWM, Hillege H, Willems TP, Ebels T, Berger RMF. Fontan Circulation over Time. Am J Cardiol. 2017 Aug 1;120(3):461-466. doi: 10.1016/j.amjcard.2017.05.005. Epub 2017 May 11.

  PMID: 28624095 BACKGROUND

• Wolff D, van de Wiel HBM, de Muinck Keizer ME, van Melle JP, Pieper PG, Berger RMF, Ebels T, Weijmar Schultz WCM. Quality of life and sexual well-being in patients with a Fontan circulation: An explorative pilot study with a mixed method design. Congenit Heart Dis. 2018 Mar;13(2):319-326. doi: 10.1111/chd.12576. Epub 2018 Mar 12.

  PMID: 29532606 BACKGROUND

Biospecimen

Retention: SAMPLES WITH DNA

Venous blood samples for biomarker analysis

Study Officials

• Tjark Ebels, MD PhD

  University Medical Center Groningen

  PRINCIPAL INVESTIGATOR

Central Study Contacts

Sophie L Meyer, BSc

CONTACT

004917681033255; s.l.meyer@umcg.nl

Study Design

• Study Type: observational
• Observational Model: COHORT
• Time Perspective: CROSS SECTIONAL
• Sponsor Type: OTHER
• Responsible Party: PRINCIPAL INVESTIGATOR
• PI Title: Prof. dr. Tjark Ebels

Study Record Dates

• First Submitted: August 15, 2018
• First Posted: September 12, 2018
• Study Start: December 3, 2018
• Primary Completion: June 1, 2019
• Study Completion: June 1, 2019
• Last Updated: September 12, 2018

Record last verified: 2018-09

Data Sharing

• IPD Sharing: Will not share