J Thorac Cardiovasc Surg 2005;129:1452-1453
© 2005 The American Association for Thoracic Surgery
Long-term survival of a patient with tetralogy of Fallot after intracardiac palliation in the pre-pump era
Takahiro Sawada, MDa,*,
Takeshi Miyairi, MDa,
Tadashi Kitamura, MDa,
Ikuo Nakai, MDa,
Haruaki Hino, MDa,
Sumio Miura, MDa,
Ikutaro Kigawa, MDa,
Sachito Fukuda, MDa,
Akihiko Sekiguchi, MDb
a Division of Cardiovascular Surgery, Mitsui Memorial Hospital, Tokyo, Japan.
b Division of Cardiovascular Surgery, National Center for Child Health and Development, Tokyo, Japan.
Received for publication November 27, 2004; accepted for publication December 7, 2004.
* Address for reprints: Takahiro Sawada, Division of Cardiovascular Surgery, Mitsui Memorial Hospital, Kanda-Izumicho 1, Kanda Izumi-cho, Chiyoda-ku, Tokyo, Japan (Email: takazawa{at}mitsuihosp.or.jp).
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Fukuda, Miyairi, Sawada, Kigawa (front row, left to right); Kitamura, Miura, Nakai, Hino (back row, left to right)
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The first successful intracardiac surgery with cardiopulmonary bypass (CPB) was performed by John H. Gibbon1 on May 6, 1953, in a patient with an atrial septal defect. The first intracardiac repair of tetralogy of Fallot (TOF) was performed by C. Walton Lillehei2 on August 31, 1954, with the use of cross-circulation. In Japan, the first cardiac surgery with CPB was a total correction of TOF performed by H. Manabe3 on April 18, 1956. We report the case of a successful direct-vision intracardiac palliation of TOF in the pre-pump era in which the patient survived for half a century.
Clinical Summary
On May 13, 1955, a 16-year-old girl with TOF underwent palliative surgery with selective cerebral hypothermia (SCH). Two cannulas were inserted into the right common carotid artery, 1 of which was inserted downward to aspirate arterial blood out of the body. The blood collected was cooled and then delivered to the brain through the other cannula, which was inserted upward. With crossclamping of both the aortic root and main pulmonary artery, accompanied by total caval occlusion, cardiac standstill was obtained by injection of acetylcholine into the right atrium. The right ventricular outflow tract (RVOT) was incised, followed by resection of an abnormal muscle bundle (AMB) and a pulmonary valvular commissurotomy. The RVOT was closed by a suture. Cardiac blood flow was stopped for 8 minutes. After the palliation, the patient remained in New York Heart Association (NYHA) class I to II status without cyanosis for a long time, living an almost normal life and being delivered of 2 children.
When the patient was 55 years old, she had shortness of breath with occasional cyanosis. When she was 65 years old, in 2004, she was admitted to the hospital in NYHA class III status because of congestive heart failure accompanied by systemic cyanosis on effort. Preoperative catheterization and echocardiography were consistent with TOF. Measured pressures were 41/12 (24) mm Hg in the pulmonary artery and 48/11 (12) mm Hg in the low-pressure chamber. The pulmonary/systemic blood flow ratio was 1.5. A double-chambered right ventricle and aneurysm of the RVOT were revealed (Figure 1). Echocardiography showed that the interchamber pressure gradient (PG) was 73 mm Hg, whereas the PG between the right atrium and high-pressure chamber was 110 mm Hg. Total correction was performed with CPB. The AMBs of the RVOT and right ventricular inflow were excised, the ventricular septal defect was closed with a Dacron patch, and the RVOT was reconstructed with a polytetrafluoroethylene patch (Gore-Tex patch; W. L. Gore & Associates, Inc, Flagstaff, Ariz). Postoperative right-sided heart failure was managed successfully. Three months after the operation the patient was in NYHA class I status with a pulmonary pressure of 29/10 (19) mm Hg and right ventricular inflow pressure of 37/11 (12) mm Hg measured by postoperative catheterization (Figure 2).
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Figure 1. Double-chambered right ventricle. An abnormal muscle bundle (AMB) separates the high pressure inflow chamber and the low pressure outflow chamber.
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Discussion
The first intracardiac surgery under SCH in Japan was performed by Kimoto and associates on January 17, 1955, in a 12-year-old boy with an atrial septal defect.4 In SCH, only the brain is cooled down to 17°C to 18°C by perfusing cold arterial blood to prevent cerebral damage during circulatory standstill. Kimoto and his colleagues found the possible circulatory standstill was 15 minutes at a cerebral temperature of 20°C.5 They reported the results of intracardiac surgery under SCH from January 1955 to April 1956. Of the 24 cases, 11 had atrial septal defects with no perioperative deaths, and 8 had TOF with 6 perioperative deaths. The patient in this case was the first of 8 cases and the first of 2 survivors. We could not find any reference in the literature to a successful correction after intracardiac palliation in the pre-pump era in elderly patients with TOF.
What was the reason for the long-term survival of our patient? The well-balanced pulmonary/systemic blood flow ratio may have been an important factor, and we speculate that the right ventricular inflow pressure, estimated to be greater than 110 mm Hg, would have prevented severe left-to-right shunt. Simultaneously, the AMB between the right ventricular inflow tract and RVOT prevented serious pulmonary hypertension. If the AMB constriction had been too severe, serious systemic cyanosis would have developed earlier because a higher right ventricular pressure would have led to a right-to-left shunt. If the constriction was small, serious pulmonary hypertension would have occurred because of the severe left-to-right shunt. In addition, because an AMB constriction existed between the right ventricular inflow tract and RVOT, the pressure in the right ventricular outflow chamber was not increased, and therefore severe congestive heart failure did not develop in the patient over a long period. In the pre-pump era, it was impossible to excise the AMB completely during the short cardiac arrest, but this inability may have resulted in the favorable outcome in this case.
References
- Gibbon Jr JH. Application of a mechanical heart and lung apparatus to cardiac surgery. Minn Med 1954;37:171-177.[Medline]
- Lillehei CW, Varco RL, Cohen M, Warden HE, Gott VL, DeWall RA, et al. The first open heart corrections of tetralogy of Fallot. A 26–31 year follow-up of 106 patients. Ann Surg 1986;204:490-502.[Medline]
- Manabe H, et al. Direct vision intracardiac surgery in man using artificial pump-oxygenator. (The first successful case in Japan) (Japanese). Rinsho Geka 1956;11:443-449.
- Asano K. Experimental and clinical studies on the open heart surgery. especially on the selective brain cooling (Japanese). Nippon Geka Gakkai Zasshi 1955;56:1150-1168.
- Kimoto S, Hayashi S. Cerebral circulation in surgery. Jpn Circ J 1956;20:312-333.
