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A 34-year-old man was referred for progressive exertional dyspnea and orthopnea. He
had end-stage restrictive cardiomyopathy with irreversible high pulmonary vascular
resistance; in 2003 at the age of 25 years, he underwent heterotopic heart transplantation
(HHT) with anastomosis of the donor pulmonary artery to the recipient right atrium.
The electrocardiogram (Fig. 1A), chest radiograph (Fig. 1B), and echocardiogram (Fig. 1C) at admission did not show major abnormalities. To investigate the potential causes
of heart failure (HF), a comprehensive hemodynamic and angiographic evaluation of
both donor and recipient hearts was performed. Right cardiac catheterization of the
donor heart (Fig. 1D), endomyocardial biopsy (Fig. 1E) performed through a 7 French (F) internal right jugular vein approach, and coronary
angiography of the recipient heart by right radial access (Videos 1 and 2; view videos online) were also unremarkable. However, left ventriculography and coronarography
of the donor heart (Fig. 1F and Video 3; view video online) showed moderate systolic dysfunction and severe cardiac allograft
vasculopathy (CAV), respectively. Anteroposterior (AP) (Fig. 1G), AP cranial (Fig. 1H) and lateral (Fig. 1I) views (Videos 4-6; view videos online, respectively) demonstrated severe in-tandem lesions of the left
anterior descending (LAD) artery and diffuse disease of a dominant left circumflex
artery (Fig. 1, G and I, arrows) with occlusion of the posterior descending artery (Fig. 1H, arrowheads). Through a right radial approach, a 6F Judkins right 4 guiding catheter
was used to obtain good backup support for the downsloping takeoff of the left coronary
artery of the donor heart. After balloon predilation, 6 everolimus-eluting stents
were implanted in the proximal-middle portion of the LAD and left circumflex arteries,
with satisfactory final results (Fig. 1, J-L and Videos 7 and 8; view videos online). The patient remained asymptomatic at 1-year clinical follow-up,
with complete recovery of left cardiac function.
Figure 1(A) A 12-lead electrocardiogram shows 2 separate and independent rhythms and electrocardiographic
complex morphologic features, the typical electrocardiographic finding after heterotopic
heart transplantation (HHT) in the absence of major abnormalities. (B) An anteroposterior chest radiographic without abnormalities illustrates the typical
radiographic chest appearance after HHT, with the donor heart located in the right
hemithorax. (C) A transthoracic echocardiogram in apical “8-chamber” view illustrates the particular
configuration of the heterotopic heart. The relationship between the recipient and
the donor hearts is also depicted. D, donor heart; R, recipient heart; RV, right ventricle;
RA, right atrium; LV, left ventricle, LA, left atrium. (D) Right cardiac catheterization of the heart performed through a 7F right internal
jugular vein approach demonstrated normal pressure values in the pulmonary circulation.
(E) Angiography in the anteroposterior projection illustrates a flexible bioptome used
to perform the endomyocardial biopsies in the recipient right ventricle through a
7F right internal jugular vein approach. (F) Left ventriculography of the donor ventricle in the anteroposterior view reveals
moderate systolic dysfunction with an ejection fraction of 40%. (G-I) Severe cardiac allograft vasculopathy (CAV) of the donor heart. (G) The anteroposterior (AP), (H) AP cranial, and (I) lateral coronary angiographic views of the donor heart demonstrate the presence
of severe CAV with critical lesions involving the left anterior descending (arrows in G and I), dominant left circumflex, obtuse marginal, and posterior descending arteries (arrowheads in H). (J-L) Final angiographic results after percutaneous coronary intervention in the donor
heart. (J) The AP, (K) AP cranial, and (L) lateral angiographic views of the donor heart show a satisfactory final angiographic
result after everolimus-eluting stents implantation in the left anterior descending
and dominant left circumflex arteries of the donor heart. PA, pulmonary artery; PWC,
pulmonary wedge capillary pressure; RA, right atrium, RV, right ventricle.