**5. Conclusion and discussion**

The patient underwent a heterotopic heart transplant with the Copeland technique [9]. The patient was treated with standard institutional immunosuppressive therapy in 1997; including, rabbit antithymocyte globulin for induction, and, then followed with cyclosporine, mycophenolate and prednisone. The pulmonary artery pressures never decreased throughout the posttransplant course. At 13 years post-transplant, the patient began to have heart failure symptoms, and was re-listed for a heterotopic heart transplant. The patient was status 1B on and died while waiting for heart transplant at almost 14 years after his heterotopic heart transplant. Of note, Al-Khaldi et al. [11] reported a case report of 22-month-old who received a heterotopic heart transplant for restrictive cardiomyopathy and severe pulmonary hypertension. The patient required sildenafil in the post-operative period due to post-operative pulmonary hypertensive crisis, and, with sildenafil the patient was weaned from ventilator support and extubated.

The second patient also had severe pulmonary hypertension of 85/30 with a mean of 48 mmHg, and, a PVR of 6 Woods units. Due to his elevated pulmonary arterial pressure, the patient was listed and had a heterotopic heart transplant. He did not have clinical right heart failure. At one-year post-transplant, the pulmonary artery pressures decreased to 39/18 with a mean of 28 mmHg. Post-transplant, he had one episode of acute rejection that required hospitalizations treated with solumedrol. In addition, he had delirium and psychosis, the steroids were decreased, and the patient improved. The patient lived well for 6 years without complications. He presented to the hospital in respiratory distress. The autopsy demonstrated

The third patient was a 36-year-old with pulmonary arterial hypertension, a dilated left ventricle with an 15% ejection fraction [a left ventricular end diastolic dimension (LVEDD) of 7.2 cm], and a slightly dilated right ventricle with preserved function and no right heart failure. His PA pressures were 69/34 mmHg with a mean of 47 with a transpulmonary gradient of 17 mmHg and a pulmonary vascular resistance of 9 Woods units. In the face of minimal evidence for right heart failure, a heterotopic heart transplant was performed. He was extubated on the first post-operative day and had normal graft function (LV ejection fraction of 64%), normal exercise tolerance, no right heart failure and a drop in his systolic PA pressure to 48 by trans-thoracic echo. Sadly, he remained impoverished and had great difficulty complying with post transplantation management. Three years later, he died of graft failure most likely from rejection.

The fourth patient was in the intensive care unit (ICU) on multiple inotropes; dobutamine, dopamine, and phenylephrine. A donor heart was accepted. The team knew the donor was "small" (5′5″ and 60 kg) compared to the 6′2.5″ and 90 kg recipient. The recipient was left awake in the operating room until the donor heart arrived. The surgeon (Jack Copeland) examined the donor heart and found it to be too small for an orthotopic transplant. The option of heterotopic placement was then discussed with the patient with a full explanation of increased risk from the size discrepancy. The recipient agreed to proceed. He survived for 11 months, leading a very active "normal" life. The patient succumbed to a recurrence of alcoholism associated with poor compliance and profound rejection. Prior to his demise, the patient had called the hospital relating symptoms of heart failure but was snowed in and unable to leave his home due to weather conditions. The patient had not taken his immuno-

a pulmonary embolus, with esophageal and gastric ulcerations.

128 Heart Transplantation

suppressive medications for several days.

Heterotopic heart transplant patients require endomyocardial biopsies as do orthotopic heart transplant recipients. Barnard first described the endomyocardial biopsy in heterotopic heart transplant patients in 1982. [12] Arouzman et al. [13] also described the use of the endomyocardial biopsy in conjunction with the Copeland heterotopic heart transplant technique [9] by leaving clips at the SVC anastomosis for visualization at the time of endomyocardial biopsy (**Figure 3**).

**Figure 3.** Endomycardial biopsy of the donor heart in a heterotopic heart transplant. Permission granted by Annals of Thoracic Surgery for reprint. Arzouman DA et al. [13].

Heterotopic heart transplantation is a valuable treatment option for patients with severe and/ or fixed pulmonary hypertension and severe pulmonary vascular resistance in the absence of native right ventricular failure. The patient cannot have an orthotopic heart transplant. Even though they may benefit from mechanical circulatory support, these patients would still benefit from a heterotopic heart transplant. The heterotopic heart transplant recipients described above lived well and had the typical post-heart transplant survival as an orthotopic heart transplant. HHT recipients must be followed as orthotopic heart transplants, have the same immunosuppression regimens and post-transplant biopsy schedule. A heterotopic heart transplant, will allow symptomatic improvement in the recipients with severe pulmonary hypertension and severely elevated PVR. Such patients may forgo LVAD implantation with attendant complications and short-term survival. Some of these patients with HHT may experience reduction of PVR while others may not such as the young heterotopic heart transplant recipient who barely had a decrease in PA pressures over the almost 14-year post-transplant course.

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[10] Copeland H, Kalra N, Gustafson M, et al. A case of heterotopic heart transplant as a "biologic left ventricular assist" in restrictive cardiomyopathy. World Journal of Pediatric and

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Based on the literature, it is difficult to determine if the heterotopic heart transplant would increase the donor pool by using size mismatched hearts. In the literature, Bleasdale et al. [4], and Newcomb et al. [5], note that the heterotopic heart transplant recipients had decreased survival compared to orthotopic heart transplant recipients. Though, the recipients in those studies were not as good candidates as the orthotopic recipients, and the heterotopic donor hearts were also considered marginal donors, often declined by other centers. Thus, the heterotopic heart transplant may still be a valuable option to increase the donor pool if the donors are not marginal and not used in less than ideal heart transplant recipients. The literature and patient review demonstrates that severe pulmonary hypertension and elevated pulmonary vascular resistance are clear indications for heterotopic heart transplantation with good survival outcomes.
