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Case Reports
. 2024 Jan 2;331(1):60-64.
doi: 10.1001/jama.2023.23823.

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction

Joseph W Turek  1 , Lillian Kang  1 , Douglas M Overbey  1 , Michael P Carboni  1 , Taufiek K Rajab  2
Affiliations
Case Reports

Partial Heart Transplant in a Neonate With Irreparable Truncal Valve Dysfunction

Joseph W Turek et al. JAMA. .

Abstract

Importance: The treatment of neonates with irreparable heart valve dysfunction remains an unsolved problem because there are no heart valve implants that grow. Therefore, neonates with heart valve implants are committed to recurrent implant exchanges until an adult-sized valve can fit.

Objective: To deliver the first heart valve implant that grows.

Design, setting, and participants: Case report from a pediatric referral center, with follow-up for more than 1 year. Participants were a recipient neonate with persistent truncus arteriosus and irreparable truncal valve dysfunction and a donor neonate with hypoxic-ischemic brain injury.

Intervention: First-in-human transplant of the part of the heart containing the aortic and pulmonary valves.

Main outcomes and measures: Transplanted valve growth and hemodynamic function.

Results: Echocardiography demonstrated adaptive growth and excellent hemodynamic function of the partial heart transplant valves.

Conclusions and relevance: In this child, partial heart transplant delivered growing heart valve implants with a good outcome at age 1 year. Partial heart transplants may improve the treatment of neonates with irreparable heart valve dysfunction.

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Conflict of interest statement

Conflict of Interest Disclosures: Dr Rajab reported receiving National Institutes of Health (NIH)/National Heart, Lung, and Blood Institute grant HL169059 (a Small Business Technology Transfer grant, in collaboration with Tissue Testing Technologies LLC, for a method to prolong storage of partial heart transplant grafts). No other disclosures were reported.

Figures

Figure 1.
Figure 1.. Partial Heart Transplant
Recipient’s anatomy with truncus arteriosus and a dysplastic, irreparable truncal valve (panel A), excision of the irreparable truncal valve (panel B), implantation of the donor aortic root with closure of the ventricular septal defect using donor tissue (panel C), and implantation of the donor pulmonary root (panel D).
Figure 2.
Figure 2.. Truncal Valve Regurgitation
The echocardiogram shows the dysplastic truncal valve with severe truncal valve regurgitation (orange jet).
Figure 3.
Figure 3.. Partial Heart Transplant Growth
The black circles show postoperative aortic and pulmonary valve annular diameters measured by echocardiography. The dotted lines show z score 0, which corresponds to the mean valve annular diameters of healthy infants with the same body surface area. The shaded areas show z scores −2 to +2, which include 95% of valve annular diameters in healthy infants with the same body surface area.
See this image and copyright information in PMC

References

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    1. Hardy WA, Kang L, Turek JW, Rajab TK. Outcomes of truncal valve replacement in neonates and infants: a meta-analysis. Cardiol Young. 2023;33(5):673-680. doi:10.1017/S1047951123000604 - DOI - PubMed
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    1. Delmo Walter EM, Huebler M, Stamm C, et al. . Adaptive growth and remodeling of transplanted hearts in children. Eur J Cardiothorac Surg. 2011;40(6):1374-1382. - PubMed

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