PMID- 24791875
OWN - NLM
STAT- MEDLINE
DCOM- 20150406
LR  - 20140505
IS  - 2542-5641 (Electronic)
IS  - 0366-6999 (Linking)
VI  - 127
IP  - 9
DP  - 2014
TI  - Surgical treatment of interrupted aortic arch associated with ventricular septal 
      defect and patent ductus arteriosus in patients over one year of age.
PG  - 1684-90
AB  - BACKGROUND: Interrupted aortic arch (IAA) is a rare congenital anomaly affecting 
      1.5% of infants with congenital heart disease. Neonatal repair of IAA is required 
      to avoid irreversible pulmonary vascular lesion. However, in China, patients with 
      IAA associated with ventricular septal defect (VSD) and patent ductus arteriosus 
      (PDA) over one year of age are common. So we investigated the outcome of surgical 
      treatment of IAA with VSD and PDA in patients over one year of age. METHODS: From 
      January 2009 to December 2012, 19 patients with IAA have undergone complete 
      single-stage repair. The patients' mean age was 4.4 years, ranging 1 to 15 years; 
      and their mean weight was 12.8 kg, ranging 4.2 to 36.0 kg. Fifteen IAA were type 
      A, four were type B. Preoperative cardiac catheterization data were available 
      from all patients. Mean pulmonary arterial pressure (mPAP) and pulmonary vascular 
      resistance (PVR) were measured. The measurements of postoperative pulmonary 
      artery pressure were taken in the operating room at the end of the case. All 
      patients underwent echocardiographic examinations before discharged from the 
      hospital. In addition, cardiac catheterization and echocardiographic examinations 
      were performed during follow-up. Selective brain perfusion through the innominate 
      artery during aortic arch reconstruction was used in all patients. Mean follow-up 
      was (1.6+/-0.8) years. RESULTS: There were two hospital deaths (2/19, 11%). One 
      patient died of pulmonary hypertension crisis, and another died of postoperative 
      low cardiac output. Five cases had other main postoperative complications but no 
      postoperative neurologic complications. Seventeen survivors were followed up, and 
      there were no late deaths or reoperation. Mean cross-clamp duration was (85+/-22) 
      minutes and selective brain perfusion duration was (34+/-11) minutes. Two patients 
      required delayed sternal closure at two days postoperatively. Intensive care unit 
      and hospital stays were (9+/-8) days and (47+/-24) days, respectively. Pressure 
      gradients across the anastomosis at most recent follow up were less than 22 mmHg. 
      mPAP regressed significantly from preoperative (62.1+/-8.1) mmHg to postoperative 
      (37.3+/-11.3) mmHg (P < 0.001) and (24.2+/-6.0) mmHg at six months after discharged 
      from the hospital (P < 0.001). The pulmonary vascular resistance also regressed 
      significantly from preoperative (1 501.4+/-335.7) dynxsxcm(-5) to (485.0+/-215.1) 
      dynxsxcm(-5) at six months after discharged from the hospital (P < 0.001). The 
      majority of the seventeen patients (89%) were in New York Heart Association 
      (NYHA) class I, and 11% remained in NYHA class II. CONCLUSIONS: Single-stage 
      repair of patients with IAA, VSD and PDA over one year of age can have good 
      surgical results and functional outcomes. Assessment and treatment of pulmonary 
      artery pressure pre-operatively and postoperatively was crucial. mPAP and 
      pulmonary vascular resistance may have regress significantly compared to 
      preoperative values.
FAU - Li, Zhiqiang
AU  - Li Z
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - Li, Bin
AU  - Li B
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - Fan, Xiangming
AU  - Fan X
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - Su, Junwu
AU  - Su J
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - Zhang, Jing
AU  - Zhang J
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - He, Yan
AU  - He Y
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China.
FAU - Liu, Yinglong
AU  - Liu Y
AD  - Department of Pediatric Heart Center, Beijing Anzhen Hospital, Capital Medical 
      University, Beijing 100029, China. Email: liucardio@163.com.
LA  - eng
PT  - Journal Article
PT  - Research Support, Non-U.S. Gov't
PL  - China
TA  - Chin Med J (Engl)
JT  - Chinese medical journal
JID - 7513795
SB  - IM
MH  - Adolescent
MH  - Child
MH  - Child, Preschool
MH  - Ductus Arteriosus, Patent/*surgery
MH  - Female
MH  - Heart Septal Defects, Ventricular/*surgery
MH  - Humans
MH  - Hypertension, Pulmonary/physiopathology
MH  - Infant
MH  - Male
MH  - Risk Factors
MH  - Treatment Outcome
MH  - Vascular Resistance/physiology
EDAT- 2014/05/06 06:00
MHDA- 2015/04/07 06:00
CRDT- 2014/05/06 06:00
PHST- 2014/05/06 06:00 [entrez]
PHST- 2014/05/06 06:00 [pubmed]
PHST- 2015/04/07 06:00 [medline]
PST - ppublish
SO  - Chin Med J (Engl). 2014;127(9):1684-90.