**4. Series of patients with heterotopic heart transplants**

**3. The heterotopic surgical technique**

donor tissue (**Figure 1**).

126 Heart Transplantation

There are two published surgical techniques for the heterotopic heart transplant. Novitzky et al. [8] published the first surgical technique pioneered by Christian Barnard in the 1970s. The heterotopic transplant technique pioneered by Barnard started with the anastomosis of the donor left atrium to the recipient left atrium. Then, the donor right atrium is anastomosed to the recipient right atrium and superior vena cava. Next, the donor aorta is sutured to the recipient aorta in an end to side fashion. The pulmonary anastomosis is the remaining anastomosis. The pulmonary artery is sutured to a dacron graft. The dacron graft is used to extend the anastomosis to the recipient pulmonary artery. Without the interposition graft, the donor and recipient pulmonary arteries would not be able to be brought together without tension or possibly not at all because of lack of length on the

In 2017, Copeland et al. [9] published an alternate heterotopic heart transplant technique as a biologic left ventricular assist. The donor heart left pulmonary veins and inferior vena cava are oversewn. The right pleura is widely opened, and the right posterior pericardium is opened toward the phrenic nerve at the level of the diaphragm, cephalad and in-between. The donor and recipient left atria are anastomosed first. Then, the donor aorta is anastomosed to the recipient aorta in an end to side fashion. The aortic cross clamp is removed, and, the patient is placed in Trendelenburg position. The donor pulmonary artery is anastomosed to the recipient right atrium. The donor superior vena cava (SVC) is anastomosed to the recipient superior vena cava in an end to side fashion (**Figure 2**). The anastomosis of the SVC is marked with clips, in order to be able to identify the anastomosis

**Figure 1.** The Barnard heterotopic heart transplant technique with an interposition Dacron Graft. Permission Granted by

Annals of Thoracic Surgery for reprint. Novitzky et al. [8].

later for endomyocardial biopsy through the right internal jugular vein.

The Copeland heterotopic heart transplant technique was used in the series of patients at the University of Arizona and University of California San Diego (by Jack Copeland). Between May 1984 to February 2011, 5 patients received a heterotopic heart transplant. The reasons for a heterotopic heart transplantation included the following: (1) fixed pulmonary hypertension with pulmonary artery (PA) pressures of 85/53 mmHg with a mean of 60 mmHg and pulmonary vascular resistance of 10 Woods units, (2) severe pulmonary hypertension of 85/30 mmHg with a mean of 48 mmHg and a PVR of 6 Woods units, (3) PA pressure of 69/34 mmHg with a trans-pulmonary artery gradient of 17 mmHg and pulmonary vascular resistance of 9 Woods units, and (4) and (5) donor recipient size mismatch in two patients.

Of the three patients with severe pulmonary hypertension, one was a 9-year old child was diagnosed with restrictive cardiomyopathy and had heart failure since early infancy [10]. The patient presented for transplant evaluation with incessant supraventricular tachycardia and pulmonary hypertension with a PA pressure of 85/53 mmHg with a mean of 60, and, a PVR of 10 Woods units. The patient's cardiac index was 3.1 l/min/sqM with an ejection fraction of 33% on echocardiogram with normal right ventricular function. The fractional shortening on the echocardiogram was 18%. The patient began to develop hepatomegaly and the total bilirubin was elevated to 2.8 mg/dl. The patient was on medical management for heart failure prior to transplant including: furosemide, spironolactone, digoxin, captopril, amiodarone, coumadin and prednisone. The patient was not on inotropic therapy. The patient was listed for heart transplantation and it was deemed that the child would not tolerate an orthotopic heart transplant because of the fixed pulmonary hypertension. The patient was not eligible for a left ventricular assist device (LVAD) because pediatric LVADs were not available in North America until 2000; when the first was implanted in North America.

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 fifth patient had a severely dilated left ventricle, with an 8 cm end diastolic dimension. He also was critically ill and was transplanted with a small donor heart (4.5 cm left ventricular end diastolic dimension (LVEDD)]. He survived for 9.5 years. As time passed his LV continued to enlarge and he developed recurrent ventricular tachycardia of the native heart accompanied by chest pain and was treated with high dose amiodarone therapy. The side effects of the amiodarone were significant including bradycardia, lethargy, and exercise intolerance. He also had blue facial discoloration. His cardiac graft function was normal on transesophageal echocardiogram. He refused relisting for orthotopic transplantation. We also offered him a left ventricle cardiectomy of the native heart and he declined. He died at home suddenly

Heterotopic Heart Transplantation

129

http://dx.doi.org/10.5772/intechopen.74582

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

**Figure 3.** Endomycardial biopsy of the donor heart in a heterotopic heart transplant. Permission granted by Annals of

9.5 years post transplantation of unknown causes.

**5. Conclusion and discussion**

Thoracic Surgery for reprint. Arzouman DA et al. [13].

biopsy (**Figure 3**).

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 a pulmonary embolus, with esophageal and gastric ulcerations.

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 immunosuppressive medications for several days.

The fifth patient had a severely dilated left ventricle, with an 8 cm end diastolic dimension. He also was critically ill and was transplanted with a small donor heart (4.5 cm left ventricular end diastolic dimension (LVEDD)]. He survived for 9.5 years. As time passed his LV continued to enlarge and he developed recurrent ventricular tachycardia of the native heart accompanied by chest pain and was treated with high dose amiodarone therapy. The side effects of the amiodarone were significant including bradycardia, lethargy, and exercise intolerance. He also had blue facial discoloration. His cardiac graft function was normal on transesophageal echocardiogram. He refused relisting for orthotopic transplantation. We also offered him a left ventricle cardiectomy of the native heart and he declined. He died at home suddenly 9.5 years post transplantation of unknown causes.
