DR.N.MADHU SANKAR*, DR.BENJANMIN NINAN, DR.K.M.CHERIAN

 Heart-Lung transplantation has come of age and is offered to patients with end-stage cardiopulmonary disorders. We have performed to Heart-Lung transplantation at the institute of Cardiovascular Diseases, Madras Medical Mission and we present a brief case history and management.

CASE 1: A 29-year-old female patient with severe primary pulmonary hypertension and severe right ventricular function was evaluated for Heart-Lung transplantation. She was suffering from acute exacerbation of breathlessness and was hospitalized frequently. She underwent successful Heart-Lung Transplantation on 3.5.99, the first of its kind in India. A 41-year-old male who was sustained intra-cerebral hemorrhage and was declared brain dead was the donor. The cold ischemic time was 2 hours and 30 minutes. She was extubated on the first postoperative day and was convalescing well. The respiratory gases were adequate and the cardiac function was good as shown by 2D echocardiography. She developed severe respiratory infection and expired on 35^th postoperative day.

CASE 2: A 31-year-old man was offered Heart-Lung transplantation after being diagnosed to have Ventricular Septal Defect with Eisenmenger syndrome. He was on regular medical follow-up and taken up for Heart-Lung transplantation upon availability of organs.

Clinically, he was cyanosed, had clubbing of fingers and a loud S2. His hemoglobin was 21 g/dl and haematocrit was 66%. The chest x-ray revealed peripheral pruning of vascular markings, a moderately enlarged heart with cardiothoracic ratio of 55%. The cardiophrenic and costophrenic angles were clear. The electrocardiogram revealed a normal sinus rhythm, rate of 106/min with a ‘p’ pulmonale and deep S in V. the electrocardiogram revealed a large muscular ventricular Septal defect with mainly right atrium and ventricle with severe pulmonary artery hypertension..

            During surgery, the lungs were found to be densely adhering to the pleural cavity. After excision of the diseased Heart-Lung block, the donor Heart-Lung block was sewn in place starting with the tracheal anastomosis followed by the right atrial suture line and the aortic suture line respectively. The heart picked up spontaneously in normal sinus rhythm. The cold ischemic time was 3 hrs and 45 min. After a brief period of support the patient was weaned of cardiopulmonary bypass with minimal isotropic supports.

            He was extubated the same evening and his immediate postoperative course was uneventful. He was started on immunosuppressive therapy with Azathioprine, Cyclosporine and steroids as also on Ganciclovir in view of the donor blood and the recipient testing positive for CMV. The histopathology report of the excised heart revealed an inlet muscular VSD with ischemic scarring and that of the lungs changes of Grade III pulmonary hypertension (Health-Edwards) with paraseptal emphysema.

            The donor Heart-Lung block was retrieved from a 40-year-old lady who was pronounced brain dead after a road traffic accident. The aorta and pulmonary artery were cannulated for infusion of cardioplegia and pneumoplegia. Prostacyclin was infused into the lungs, ante grade crystalloid cardioplegia delivered to the aortic root followed by pneumoplegia.

HISTORY :

            The beginning of cardiac surgery and the transplantation of vascularized organs can be traced to the pioneering work of Alexis Carrel. In a description of early experimental transplantation of  a puppy’s heart into the neck of an adult dog, he mentions another experiment where he transplanted the lungs together with the heart. Dramatic experiments were performed in the late 1940s by Demikhov. “Damka” the dog lived for six days after Heart-Lungs transplantation. The next report of experiments on heart and lungs replacement are credited to Neptune and Marcus et al respectively (1953). Lower and Shumway, Sen, Longmore and Nakae et al contributed further. Three Heart-Lung transplants were performed by cooley (1968), Lillehei (1969) and Barnard (1971). Primate studies were begun by Rritz and colleagues at the Stanford University, California, in 1978 and they performed the first successful human Heart-Lung transplant in 1982.

CLASSICAL INDICATIONS FOE HEART-LUNGS TRANSPLANTATION:

1. Eisenmenger syndrome
2. Primary pulmonary hypertension
3. Cystic fibrosis
4. Fibrosing alveolitis
5. Emphysema
6. Sarcoidosis
7. Bronchiectasis
8. Histiocytosis – X
9. Pulmonary fibrosis

Currently, for majority of these indications, Heart-Lung transplant has given way to Bilateral Sequential Lung Transplantation. Thus for Heart- Lung Transplantation, present day indications are Eisenmenger syndrome with complex congenital anomalies, primary pulmonary hypertension with severe RV dysfunction and proximal bronchiectasis i.e those involving mainstem bronchi.

            The rate limiting factor in clinical Heart-Lung transplantation is the continuing and acute shortage of suitable donor organs. The problem is particularly acute in the case of Heart-Lung donors because of the propensity for neurogenic pulmonary oedema, atelectasis, pneumonia and other pulmonary complications in brain-dead, ventilated patients. Due to the strict criteria for accepting lungs for transplantation, approximately only 15% of cardiac donors are suitable for heart-lung Transplantation.

IMMUNOSUPPRESSION: T he introduction of cyclosporine₂  in the 1980s, revolutionized Heart and Heart – Lung transplantations. The immunosuppressive regimen differs from orthotopic heart transplantation in that steroids are minimized to enhance tracheal healing. A typical regimen includes Cyclosporine and Azathioprine.

REJECTION: Cardiac and pulmonary rejection can occur asynchronously. Cardiac rejection is graded on the same international grading scale as for isolated cardiac rejection. The diagnosis of pulmonary rejection is more difficult and subtle to ascertain than cardiac rejection. Rejection is treated with a pulse of steroids. The rate of cardiac rejection in heart-lung Transplantation recipients is significantly less that seen in patients receiving only heart.

LONG – TERM RESULTS: Combined Heart-Lung transplantation is a proven technique. International accumulated data from the Registry of the International Society for Heart and Lung transplantation documents a 1-year survival of approximately 60% in all recipients and a 5-year survival of 40-50%. The survival at 11 years is approximately 21%

CONCLUSION: Heart – Lung transplantation is an extremely effective treatment for combined end-stage cardiac and pulmonary disease. Survival rates in excess of 70% after 1 year can be anticipated in healthy young patients.

            Donor shortage is the rate limiting factor. In view of its efficacy, it should be offered to appropriate recipients who have no hope otherwise.