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Asian Cardiovasc Thorac Ann 2003;11:250-254
© 2003 Asia Publishing EXchange Ltd


ORIGINAL CONTRIBUTION

One-Stage Repair of Cardiac and Arch Anomalies Without Circulatory Arrest

Kona Samba Murthy, MCh, Robert Coelho, MCh, Christopher Roy, MCh, Snehal Kulkarni, DNB, Benjamin Ninan, MD, Kotturathu Mammen Cherian, FRACS

Institute of Cardiovascular Diseases, Madras Medical Mission, Mogappair, Chennai, India

For reprint information contact: Kona Samba Murthy, MCh Tel: 91 44 2656 5961/5968 Fax: 91 44 2656 5859 email: konasmurthy{at}hotmail.com Institute of Cardiovascular Diseases, Madras Medical Mission, 4A Dr. JJ Nagar, Mogappair, Chennai 600 050, India.


    ABSTRACT
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Between 1999 and 2002, 23 patients underwent single-stage complete repair of cardiac anomalies and aortic arch obstruction, without circulatory arrest. Median age was 1.2 years. Intracardiac defects included ventricular septal defect in 9, double-outlet right ventricle in 6, d-transposition of the great arteries and ventricular septal defect in 2, subaortic obstruction in 3, and atrial septal defect in 3. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic aortic arch, and 3 had interrupted aortic arch. Simple techniques were employed such as cannulation of the ascending aorta near the innominate artery and maintaining cerebral and myocardial perfusion. After correction of arch obstruction, intracardiac repair was undertaken. The mean cardiopulmonary bypass time was 169 min, aortic crossclamp time was 51 min, and arch repair took 16 min. There was no operative mortality or neurological deficit. In follow-up of 1–43 months, no patient had residual coarctation. This simplified technique avoids additional procedures, reduces ischemic time, and prevents problems related to circulatory arrest.


    INTRODUCTION
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Intracardiac defects associated with arch obstruction is a complex congenital anomaly. The optimal management of such malformation remains controversial. Initially, a two-stage operation was described, which included repair of the arch obstruction through a left thoracotomy, with or without pulmonary artery banding, and subsequent intracardiac repair through a median sternotomy.1,2 Recently, an aggressive approach has been adopted with single-stage repair through a median sternotomy; this can be performed with or without circulatory arrest.3–8 Various reports have described the adverse effects of total circulatory arrest, and the current trend is to avoid it whenever possible.9 Our experience of the ease of exposing the aortic arch and descending thoracic aorta through a median sternotomy prompted us to perform single-stage repair of arch obstruction and intracardiac anomalies, without circulatory arrest.10


    PATIENTS AND METHODS
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
From June 1999 to December 2002, 23 patients underwent single-stage repair of intracardiac anomalies associated with arch obstruction. Their ages ranged from 1 month to 3 years (median 1.2 years) and weights ranged from 2.2 to 12 kg (median, 5.5 kg). The clinical spectrum included ventricular septal defect (VSD) in 9 patients, double-outlet right ventricle and VSD in 6, d-transposition of the great arteries with VSD in 2, subaortic obstruction in 3, ostium secundum atrial septal defect (ASD) in 2, and ostium premium ASD in 1. Fourteen patients had coarctation of the aorta, 6 had coarctation with hypoplastic arch, and 3 had interrupted aortic arch.

Arterial pressure monitoring lines were placed in the right radial and dorsal pedis or femoral arteries. After a routine median sternotomy, the thymus was partly excised, the aortic arch with its branches and duct were dissected and mobilized to a sufficient length to achieve a tension-free anastomosis. The neck vessels were looped and snares were passed around them. The arterial cannula was inserted into the distal ascending aorta (close to the innominate artery) or into the innominate artery. Using bicaval venous cannulation, cardiopulmonary bypass was established, and the patient was cooled to 28°C.

For repair of coarctation of the aorta, the ductus was ligated and divided before starting to cool the patient. During cooling, the descending thoracic aorta was completely mobilized without sacrificing the intercostal arteries. A vascular clamp was applied to the proximal aorta distal to the left subclavian artery, and another clamp was applied to the descending aorta distal to the coarcted segment. During aortic clamping, the pump flows were reduced to 30%–50% of the level required to achieve right radial artery mean pressure of 40–60 mm Hg. The coarcted aortic segment was excised along with the ductal tissue to obtain a wide diameter at both aortic ends. End-to-end anastomosis was performed with 6/0 polypropylene continuous suture. After completion of the anastomosis, the distal vascular clamp was released. The neck vessels were snared tightly temporarily, a needle was inserted into the proximal part of the arch for de-airing, and the proximal vascular clamp was released (Figure 1Go). After establishing flow in the descending aorta, the neck vessel snares were released and flows increased to normal levels. Under cold blood cardioplegic arrest, the intracardiac repair was carried out as usual.




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Figure 1. Repair of coarctation of aorta. (A) Clamping the coarctation segment, the dashed lines indicate resection of the coarctation. (B) After completion of coarctation repair.

 
For repair of coarctation with hypoplastic arch, the descending thoracic aorta was mobilized extensively across the entire length. The left common carotid and left subclavian arteries were snared. Isolated cerebral and myocardial perfusion was established by clamping the aortic arch close to the origin of the innominate artery, with an angled clamp. The pump flows were reduced to maintain the right radial artery pressure at 40–60 mm Hg. Another clamp was placed in the descending aorta distal to the coarctation. The coarcted segment and the ductal tissue were excised so that the distal aorta had a beveled end. A longitudinal incision was made along the undersurface of the arch, and arch reconstruction was undertaken using a 6/0 polypropylene continuous suture. During the anastomosis, both vascular clamps were brought close together to prevent anastomotic tension. After completion of the anastomosis, the distal clamp was released, pump flows were reduced, and all neck vessels were snared temporarily. A de-airing needle was inserted into the arch and the proximal clamp was released (Figure 2Go). After adequate de-airing, the neck vessel snares were released and flows were increased to normal levels. Subsequently, under cardioplegic arrest, the required intracardiac repairs were carried out.





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Figure 2. Repair of coarctation with hypoplastic arch. (A) Clamping the aortic arch along with the coarctation (dashed lines indicate resection of the coarctation segment). (B) After excising the coarctation segment and incising the undersurface of the arch. (C) After completion of arch repair.

 
For repair of interrupted aortic arch, the branches of both pulmonary arteries were snared in addition to the neck vessels. After establishing cardiopulmonary bypass, the pulmonary arterial branches were occluded and cooling was started slowly. In this situation, the duct was not ligated as described earlier. Circulation in the lower part of the body and spinal cord was maintained via the ductus arteriosus from the right ventricular ejection. The pressure in the lower part of the body was recorded by the dorsalis pedis or femoral arterial line. Patients were cooled to 28°C–30°C to keep the heart beating and achieve hypothermia of the spinal cord, liver, kidney, intestine, and other distal organs. The patent ductus arteriosus (PDA) was divided between the clamps, and the pulmonary arterial snares were released. A clamp was placed on the distal arch and another clamp was placed in the descending aorta. The PDA segment was resected and end-to-end anastomosis was performed. After reducing the flows, the neck vessels were occluded and the clamps were released (Figure 3Go). De-airing was carried out and the neck vessel snares were released. The ductal end of the pulmonary artery was repaired separately.




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Figure 3. Repair of interrupted aortic arch. (A) Dashed lines indicate resection of the arch, descending aorta, and patent ductus arteriosus (for clarity, vascular clamps are not shown). (B) After completion of interrupted arch repair.

 

    RESULTS
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
Cardiopulmonary bypass times ranged from 135 to 232 min (mean, 169 min) and aortic crossclamp times ranged from 26 to 84 min (mean, 51 min). In 14 patients including those with double-outlet right ventricle, VSD closure was accomplished. An arterial switch operation with VSD closure was performed in 2 patients. Subaortic resection was carried out in 3 patients. Two patients underwent secundum ASD closure, and there was one case of ostium primum ASD closure with mitral valve repair. The intracardiac repairs were completed under routine cardioplegic arrest. Repair of coarctation was performed in 14 patients, coarctation and arch repair in 6, and interrupted arch repair in 3. Time taken for arch repair varied from 8 to 32 min (mean, 16 min). Tissue-to-tissue anastomosis was achieved in all patients for repair of arch obstruction without the use of any prosthetic material, to achieve good future growth in these children. There was no operative mortality. Follow-up ranged from 1 to 43 months (median, 22 months). The duration of postoperative ventilation was 1–4 days, and intensive care unit stay was 2–6 days. No patient had residual coarctation or underwent reoperation. There was no late death.


    DISCUSSION
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 
There are increasing reports that single-stage repair is as good as, or even better than, a two-stage approach.2–11 The optimal repair of aortic obstruction, with or without circulatory arrest, remains controversial. Although deep-hypothermic circulatory arrest provides significant protection from brain damage, it may be associated with transient cerebral dysfunction and delayed psychomotor development.9 It also prolongs the myocardial ischemic time. To avoid prolonged circulatory arrest, Ishino and colleagues8 employed an isolated cerebral myocardial perfusion technique for single-stage repair through a polytetrafluoroethylene tube graft sutured to the innominate artery. In our technique, by cannulating the distal part of the ascending aorta (at the origin of the innominate artery) or the innominate artery, and placing an angled clamp close to it, one can achieve both myocardial and cerebral perfusion during arch reconstruction. It definitely avoids the cumbersome tube graft anastomosis to the innominate artery. Also, there is no need for special instruments or material to perform this procedure. The conventional method for repair of interrupted aortic arch was to cannulate both the ascending aorta and main pulmonary artery or the PDA, to perfuse both upper and lower parts of the body.11 This is cumbersome and time consuming, therefore, we developed a way of perfusing the upper body by conventional cannulation of the ascending aorta, and the lower body through the PDA from right ventricular ejection, after snaring both pulmonary artery branches. The vital organs were cooled to 28°C–30°C, at which they can tolerate 20–30 min of ischemia reasonably well. Recurrence of coarctation after repair has been mainly attributed to growth failure of the anastomosis, inadequate resection of ductal tissue, or the persistence or development of a coarctation shelf.12–14 Several histologic examinations have shown the presence of circumferential ductal tissue extending in the area of residual coarctation.15–17 Late aneurysm formation is another widely recognized disadvantage of subclavian flap and synthetic patch angioplasty.18,19 Moreover, shortening of the arm, claudication, and subclavian steel syndrome may also occur.20

To avoid delayed recurrence of arch obstruction, we performed extended aortoplasty as described by Conte and colleagues4 who highlighted 3 factors for achieving restoration of the normal aortic arch: wide dissection and mobilization of the entire thoracic aorta; a large resection of the coarctation segment including all ductal tissue; and incision of the arch concavity extending beyond the origin of the left common carotid artery, followed by anastomosis. In our series, we achieved tissue-to-tissue anastomosis without the use of prosthetic material, to encourage future growth in these children. There was no need for additional cannulation of the pulmonary artery or PDA, due to our method of lower body perfusion through the PDA with the pulmonary artery branches snared during cooling to protect the target organs.

Repair of these arch anomalies under cardiopulmonary bypass was less risky compared to the thoracotomy approach for staged repair. This simplified technique avoids additional procedures and complicated paraphernalia. It decreases myocardial ischemic time and avoids problems related to circulatory arrest. By reducing the number of operations and hospitalization, and it has also proved more economical. Early and midterm results are encouraging.


    Acknowledgments
 
We thank Mr Dhanabalan, artist, and Mr Pary Uma, medical illustrator, for their help in preparing the manuscript.


    REFERENCES
 TOP
 ABSTRACT
 Introduction
 Patients and Methods
 Results
 Discussion
 References
 

  1. Park JK, Dell RB, Ellis K, Gersony WM. Surgical management of the infant with coarctation of the aorta and ventricular septal defect. J Am Coll Cardiol 1992;20:176–80.[Abstract]

  2. Quaegebeur JM, Jonas RA, Weinberg AD, Blackstone EH, Kirklin JW. Outcomes in seriously ill neonates with coarctation of the aorta. A multiinstitutional study. J Thorac Cardiovasc Surg 1994;108:841–54.[Abstract/Free Full Text]

  3. DeLeon SY, Downey FX, Baumgartner NE, Ow EP, Quinones JA, Torres L, et al. Transsternal repair of coarctation and associated cardiac defects. Ann Thorac Surg 1994;58:179–84.[Abstract]

  4. Conte S, Lacour-Gayet F, Serraf A, Sousa-Uva M, Bruniaux J, Touchot A, et al. Surgical management of neonatal coarctation. J Thorac Cardiovasc Surg 1995;109:663–75.[Abstract/Free Full Text]

  5. Gaynor JW, Wernovsky G, Rychik J, Rome JJ, DeCampli WM, Spray TL. Outcome following single-stage repair of coarctation with ventricular septal defect. Eur J Cardio-thorac Surg 2000;18:62–7.[Abstract/Free Full Text]

  6. Sano S, Mee RBB. Isolated myocardial perfusion during arch repair. Ann Thorac Surg 1990;49:970–2.[Abstract]

  7. Asou T, Kado H, Imoto Y, Shiokawa Y, Tominaga R, Kawachi Y, et al. Selective cerebral perfusion technique during aortic arch repair in neonates. Ann Thorac Surg 1996;61:1546–8.[Abstract/Free Full Text]

  8. Ishino K, Kawada M, Irie H, Kino K, Sano S. Single-stage repair of aortic coarctation with ventricular septal defect using isolated cerebral and myocardial perfusion. Eur J Cardio-thorac Surg 2000;17:538–47.[Abstract/Free Full Text]

  9. Greeley WJ, Kern FH, Ungerleider RM, Boyd JL, Quill T, Smith LR, et al. The effect of hypothermic cardiopulmonary bypass and total circulatory arrest on cerebral metabolism in neonates, infants and children. J Thorac Cardiovasc Surg 1991;101:783–94.[Abstract]

  10. Murthy KS, Krishnanaik S, Coelho R, Punnoose A, Arumugam SB, Cherian KM. Median sternotomy single stage complete unifocalization for pulmonary atresia, major aortopulmonary collateral arteries and VSD: early experience. Eur J Cardio-thorac Surg 1999;16:21–5.[Abstract/Free Full Text]

  11. Karl TR, Sano S, Brawn W, Mee RBB. Repair of hypoplastic or interrupted aortic arch via sternotomy. J Thorac Cardiovasc Surg 1992;104:688–95.[Abstract]

  12. Ibarra-Perez C, Castaneda AR, Varco RL, Lillehei C. Recoarctation of the aorta: nineteen years clinical experience. Am J Cardiol 1969;23:778–84.[Medline]

  13. Korfer R, Meyer H, Kleikamp G, Bircks W. Early and late results after resection and end-to-end anastomosis of coarctation of the thoracic aorta in early infancy. J Thorac Cardiovasc Surg 1985;89:616–22.[Abstract]

  14. Sanchez GR, Balsara RK, Dunn JM, Mehta AV, O’Riordan AC. Recurrent obstruction after subclavian flap repair of coarctation of the aorta in infants: can it be predicted or prevented? J Thorac Cardiovasc Surg 1986;89:128–35.

  15. Ho SY, Anderson RH. Coarctation, tubular hypoplasia, and the ductus arteriosus: histological study of 35 specimens. Br Heart J 1983;49:317–23.[Abstract/Free Full Text]

  16. Elzenga NJ, Gttenberger De Groot AC. Localized coarctation of the aorta: an age-dependent spectrum. Br Heart J 1983;49:317–23.

  17. Russell GA, Berry PJ, Watterson K, Dhasmana JP, Wisheart JD. Patterns of ductal tissue in coarctation of the aorta in the first three months of life. J Thorac Cardiovasc Surg 1991;102:596–601.[Abstract]

  18. Martin MM, Beekman RH, Rocchini AP, Crowley DC, Rosenthal A. Aortic aneurysm after subclavian angioplasty repair of coarctation of the aorta. Am J Cardiol 1988;61:951–3.[Medline]

  19. Del Nido PJ, Williams WG, Wilson GJ, Coles JG, Moes CA, Hosokawa Y, et al. Synthetic patch angioplasty for repair of coarctation of the aora: experience with aneurysm formation. Circulation 1986;74(Suppl I):I32–6.

  20. Todd PJ, Dangerfield PH, Hamilton DL, Wilkinson JL. Late effects on the left upper limb of subclavian flap aortoplasty. J Thorac Cardiovasc Surg 1983;85:678–87.[Abstract]





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