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Case Report
Acta Cardiol Sin 2005;21:54-7
Complete Repair of Interrupted Aortic Arch with
Ventricular Septal Defect in a Premature
Weighing 1600 Grams ¾ A Case Report
Jun-Yen Pan,*1 Kai-Sheng Hsieh,2 Pei-Leun Kang,1 Anna Lo,1 Tung-Ho Wu1 and Kwok-Kei Cheng1
A premature neonate with gestational age of 32 weeks and birth body weight of 1650 grams was diagnosed to have
type A interrupted aortic arch, perimembranous type ventricular septal defect, atrial septal defect, and patent
ductus arteriosus. One-stage complete reconstruction of aortic arch and concomitant repair of intracardiac defects
was performed at age of 13 days with body weight of 1600 grams. Through innominant artery and retrocardiac
descending aorta cannulation for continuous cerebral and lower body perfusion, deep hypothermic circulatory
arrest was avoided. Postoperative renal function was excellent, and there were no neurological sequelae after 24
months’ follow-up.
Key Words:
Premature · Interrupted aortic arch · Ventricular septal defect
INTRODUCTION
nate with low body weight who underwent primary
one-stage repair of interrupted aortic arch and intracardiac
lesions without circulatory arrest during operation.
Low birth weight is a risk factor for poor outcome in
corrective surgery for many cardiac defects.1 Congenital
cardiac defects in low birth weight infants are traditionally managed with supportive therapy or palliative
operations, followed by a definitive repair later at a more
conventional age and body weight. The morbidity with
such approach is high. In addition, there is also a major
concern among neonatologists regarding intracranial
bleeding after cardiopulmonary bypass. Interrupted aortic
arch is a severe form of cardiac anomaly with nearly 100
percent mortality if not recognized and treated early. The
associated intracardiac lesions often lead to death if only
the arch anomaly is repaired. We report a premature neo-
CASE REPORT
A 6-day-old neonate was transferred from a local
hospital because of prematurity with gestational age of
32 weeks. Birth body weight was 1650 grams. He was
born with vaginal deliver, and the Apgar scores were 7
and 8 at 1 minute and 5 minutes, respectively. After
birth, shortness of breath was noted and grade I respiratory distress syndrome (RDS) was impressed. Respiratory
support with nasal continuous positive airway pressure
was given. Physical examinations revealed poor activity
with coarse breat sounds, and grade II/VI continuous
murmur was heard over the left upper sternal border. The
chest roentgenography showed cardiomegaly and
groundglass opacity over bilateral lung fields. ECG
showed sinus tachycardia, with heart rate between
140-160 beats per minute. Transthoracic echocardiography
(TTE) showed type A interrupted aortic arch (IAA),
perimembranous type ventricular septal defect (VSD),
Received: April 7, 2004
Accepted: June 4, 2004
1
Division of Cardiovascular Surgery, 2Department of Pediatrics,
Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan.
Address correspondence and reprint requests to: Dr. Jun-Yen Pan,
Division of Cardiovascular Surgery, Kaohsiung Veterans General
Hospital, No. 386 Ta-Chung 1st Road, Kaohsiung 813, Taiwan, R.O.C.
Tel: 886-7-342-2121 ext. 3039; Fax: 886-7-342-0956;
E-mail: [email protected]
Acta Cardiol Sin 2005;21:54-7
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Complete Repair of Interrupted Aortic Arch and Ventricular Septal Defect
cannula (Edwards Lifesciences, Irvine, CA, USA) and
14Fr. RMI cannula (Edwards Lifesciences, Irvine, CA,
USA), respectively (Figure. 1A). Under high flow (cardiac index 3.0-3.5 L/M2BSA) and moderate hypothermic
(rectal temperature 26 °C) cardiopulmonary bypass, the
PDA was divided with ductal tissue excised. Aortic
arch reconstruction and intracardiac repair were performed under cold crystalloid cardioplegic arrest.
Direct anastomosis between ascending and descending
aorta with continuous 6-0 polydioxanone suture in an
end-to-side fashion was performed because of aortic
arch hypoplasia, which was only 2.0 mm in diameter
(Figure. 1B). VSD was repaired with Teflon patch anchoring with 6-0 polypropylene sutures interruptedly.
Aortic cross-clamp time was 81 minutes, and cardiopulmonary bypass time was 231 minutes. No circulatory
arrest was used during operation. Postoperative TTE
showed no residual VSD or ASD, and only mild residual
aortic stenosis at the anastomotic site, with pressure gradient of less than 20 mmHg. Brain sonography showed
no intraventricular hemorrhage. Neurological examination showed no neurological deficit. Renal function was
within normal limits throughout the postoperative
course. After follow-up for 24 months, the patient grew
well without any intervention.
atrial septal defect (ASD), and patent ductus arteriosus
(PDA) with right-to-left shunt. Prostaglandin E was infused after diagnosis. Operation was performed at the
age of 13 days with body weight 1600 grams. Standard
median sternotomy with partial thymectomy was performed. Arterial cannulation was made with an 8Fr. DLP
cannula (Medtronic Inc., Minneapolis, MN, USA) through
a 3.5-mm expanded-polytetrafluoroethylene (E-PTFE)
graft connected to the innominant artery for cerebral
perfusion, and a 6Fr. DLP cannula (Metronic Inc., Minneapolis, MN, USA) in the retrocardiac descending
aorta for lower body perfusion. Venous drainage was
obtained by bicaval cannulation with 8Fr. RMI angle
DISCUSSION
Recent advances in neonatal cardiac surgery have
dramatically improved the outcomes of surgery in the
past decade. However, low birth weight is still considered by many as a contraindication to cardiopulmonary
bypass and anatomic repair of even relatively simple cardiac defects. In recent years, some centers reported
excellent results after early complete repair of congenital
cardiac defects in low birth weight and very low birth
weight premature patients. 2,3 Pawade and associates 2
reported on a series of 60 patients with low birth weight
who underwent operations for various congenital heart
defects, with a 16.5% early mortality rate and a 13.3%
late mortality rate. In their experience, the risk factors
for early death after the operation in patients smaller
than 2500 grams were preoperative metabolic acidosis,
univentricular palliation, duration of cardiopulmonary
bypass, and postoperative low cardiac output. Reddy
Figure 1. (A) Cannulation at innominante artery by means of E-PTFE
graft for cerebral perfusion and direct descending aorta cannulation for
lower body perfusion. E-PTFE = expanded polytetrafluoroethylene;
Innom A = innominate artery; Des Ao = descending aorta; MPA = main
pulmonary artery; PDA = patent ductus arteriosus. (B) Direct
anastomosis between ascending aorta and descending aorta due to
aortic arch hypoplasia.
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Acta Cardiol Sin 2005;21:54-7
Jun-Yen Pan et al.
and associates 3 reported on a series of 102 infants no
larger than 2500 grams with a 10% early mortality rate
and 9% late mortality rate. They believed delay in intervention was associated with increased complications
including ventilatory dependency, failure to thrive, absence of weight gain, sepsis, chronic pulmonary disease,
necrotizing enterocolitis, and acute renal failure.
IAA is a rare congenital heart disease in which the
aortic arch is atretic or interrupted.4 The prognosis for
untreated infants is extremely poor, and survival beyond
infancy is uncommon. The surgical approach to this condition in different centers varies considerably. Most
reports advocate two-stage repair,5,6 while other centers
prefer a one-stage repair of the defect and associated
anomalies.4,7,8 In recent years, the results of both primary
and two-stage repair of IAA have improved, and the
early mortality in some centers is about 10%.4,6 In the
majority of cases, the mortality and morbidity after surgical repair are a result of total preoperative and
intraoperative ischemia (operative circulatory arrest
time) and systemic or organ-related failure (low cardiac
output syndrome, sepsis, acidosis, coagulopathy, renal,
hepatic or multi-organ failure syndrome).8 Our patient
was a case of prematurity with gestational age of 32
weeks and birth body weight only 1650 grams. He had
severe congenital cardiac defects, with type A IAA, aortic arch hypoplasia, VSD, ASD, and PDA. We used the
policy of early intervention and one-stage complete repair. During operation, we cannulated the innominant
artery through a 3.5-mm E-PTFE graft for cerebral perfusion and retrocardiac descending aorta just superior to
the diaphragm for lower body perfusion. Arch reconstruction and repair of VSD and ASD were performed
under continuous perfusion to the brain and lower body.
Postoperative renal function was excellent, and there
was no neurological deficit. The technique of descending
aorta cannulation for aortic arch reconstruction was first
reported by Imoto and associates,9 and was believed to
be an effective method to avoid circulatory arrest which
Acta Cardiol Sin 2005;21:54-7
had been proved to have detrimental effects on renal and
neurologic function. During the short-term follow up of
24 months, the patient was doing well and grew well.
The long term result needs to be further evaluated.
In summary, one-stage repair of congenital heart defects can be performed safely and effectively in premature
and low birth weight infants, as in the patient we have
reported herein. Special cannulation technique as we
mentioned above to avoid circulatory arrest may be a
useful alternative in aortic arch reconstruction surgery.
REFERENCES
1. Kirklin JK, Blackstone EH, Kirklin JW, et al. Intracardiac surgery
in infants under 3 months: incremental risk factors for hospital
mortality. Am J Cardiol 1981;48:500-6.
2. Pawade A, Waterson K, Laussen P, et al. Cardiopulmonary bypass
in neonates weighing less than 2.5 kg: analysis of the risk factors
for early and late mortality. J Card Surg 1993;8:1-8.
3. Reddy VM, McElhinney DB, Sagrado T, et al. Results of 102
cases of complete repair of congenital heart defects in patients
weighing 700 to 2500 grams. J Thorac Cardiovasc Surg 1999;
117(2):324-31.
4. Jonas RA, Quaegebeur JM, Kirklin JW, et al. Outcomes in
patients with interrupted aortic arch and ventricular septal defect:
multi-institutional study. Congenital Heart Surgeons’ Society. J
Thorac Cardiovasc Surg 1994;107:1099-109.
5. Irwin ED, Braunlin EA, Foker JE. Staged repair of interrupted
aortic arch and ventricular septal defect in infancy. Ann Thorac
Surg 1991;52:632-9.
6. Sawin RS, Hall DG, Mansfield PB, Rittenhouse EA. Staged
repair of interrupted aortic arch with ventricular septal defect
compared with primary repiar in infancy. Am J Surg 1989;
157:487-9.
7. Davies MJ, Dyamenahalli U, Leanage RR, Firmin RK. Total
one-stage repair of aortopulmonary window and interrupted aortic
arch in a neonate. Pediatr Cardiol 1996;17:122-4.
8. Tlaskal T, Hucin B, Hruda J, et al. Results of primary and
two-stage repair of interrupted aortic arch. Eur J Cardiothorac
Surg 1998;14:235-42.
9. Imoto Y, Kado H, Shiokawa Y, et al. Norwood procedure without
circular arrest. Ann Thorac Surg 1999;68:559-61.
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Case Report
Acta Cardiol Sin 2005;21:54−7
1600 公克早產兒主動脈弓斷離合併心室中膈缺損之
完全矯正 — 一病例報告
潘俊彥* 1 謝凱生 2 康沛倫 1 盧麗芬 1 吳東和 1 鄭國琪 1
高雄市 高雄榮民總醫院 心臟外科1 小兒部2
一例 32 週早產兒出生體重 1650 公克，診斷為 A 型主動脈弓斷離，心室及心房中膈缺損，
開放性動脈導管。病患於 13 天大體重 1600 公克時接受一期主動脈弓重建及心內修補手術，
藉由無名動脈及心後下行主動脈持續灌注，深低體溫循環停止可以完全避免。術後病患腎
功能正常，且無神經學方面之缺損。
關鍵詞：早產兒、主動脈弓斷離、心室中膈缺損。
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