ABSTRACT
We describe two cases with pulmonary atresia, intact ventricular septum (PA/IVS), and right ventricle-dependent coronary circulation, who suffered from myocardial ischemic symptoms. Case 1 : A female infant diagnosed with PA/IVS during the fetal period was born after 40 weeks of gestation, weighing 3,078 g. Aortography demonstrated that the right coronary artery (RCA) was interrupted at the proximal segment, and the left coronary artery (LCA) did not show any stenosis. Right ventriculogram revealed sinusoidal connections filling the distal part of the right coronary artery and fistulous communications to the left anterior descending artery. Temporary ST segmental depression in ECG during milk feeding or crying improved spontaneously with time. Bidirectional Glenn shunt (BDG) was performed at 3 months old, and she underwent Fontan repair at 4 years old. She is doing well with no evidence of myocardial ischemic change. Case 2 : A female infant was born after 39 weeks of gestation, weighing 3,062 g. Aortography demonstrated that the RCA was interrupted at the middle segment, and the distal area depended on the right ventricle. LCA ostium at the ascending aorta was absent. Multiple sinusoidal connections were seen in the right ventricular myocardium with filling of the LCAs but no reflux into the aortic sinuses. After ventricular tachycardia occurred at 2 months old, left ventricular function deteriorated. BDG with ligation of the arterial duct was performed at age 3 months. The arterial blood pressure was 76/53 (62) mmHg and pressure in SVC was 21 mmHg immediately after the operation. However, arterial oxygen saturation, right atrium venous oxygen saturation and left ventricular ejection fraction were 50%, 16%, and 10%, respectively. Although extracorporeal membrane oxygenation was established for circulatory and myocardial oxygenation support on the postoperative day 2, her left ventricular function did not recover and she died on postoperative day 5. It is difficult to rescue such a case in which almost all coronary circulation depends on the right ventricle filled with desaturated blood.
ABSTRACT
ObjectiveTo investigate the clinical efficacy of bidirectional Glenn shunt with noncardiopulmonary bypass(CPB) for treating congenital heart disease.MethodsThe clinical data of forty-four patients receiving bidirectional Glenn shunt from August 2005 to August 2011 were analyzedretrospectively,twenty-three cases of which were treated by bidirectional Glenn shunt with non-CPB (non-CPB group) and twenty-one with CPB (CPB group).The duration of ventilator-assisted breathing,chest drainage volume,amount of blood product usage,postoperative hospital stay and pulse oxygen saturation (SpO2) before leaving the hospital were compared and analyzed between two groups.ResultsForty-four patients were all cured.The duration of ventilator-assisted breathing,chest drainage volume and the amount of blood product usage of non-CPB group were less than those of CPB group [ (4.5 ± 1.3 ) h vs.(6.7 ± 1.5 ) h,(4.6 ± 1.4 ) ml/kg vs.(7.7 ± 3.1 ) ml/kg,( 221.8 ± 97.6 ) ml vs.( 423.4 ± 149.7 ) ml,P ＜ 0.05 ].There was no significant difference of the postoperative hospital stay and SpO2 before leaving the hospital between non-CPB group and CPB group[ (7.4 ± 2.3 ) d vs.(7.8 ± 1.9) d,0.91 ± 0.05 vs.0.88 ± 0.39,P ＞ 0.05 ].Conclusion Bidirectional Glenn shunt with non-CPB is a safe and feasible surgical approach,which is worthy of promotion.
ABSTRACT
ObjectiveTo evaluate the clinical application of bidirectional Glenn shunt without extracorporeal circulation(ECC) on treatment of children with complicated congenital heart disease (CHD).MethodsForty-six patients with complicated CHD(without ECC group) underwent bidirectional Glenn shunt without ECC,and 40 patients with complicated CHD (with ECC group) underwent bidirectional Glenn shunt with ECC.The therapeutic effect was compared between two groups.ResultsThere was no operative mortality in two groups.The pulmonary artery pressure in without ECC group was significantly lower than that in with ECC group [( 16.7 ± 1.2) mm Hg ( 1 mm Hg =0.133 kPa) vs.( 18.9 ± 1.0) mm Hg,t =4.686,P=0.026 ].Duration of respirator assistance after operation in without ECC group was significantly lower than that in with ECC group [ ( 12.2 ± 2.7) h vs.( 19.2 ± 2.8) h,t =2.972,P =0.041 ].There was no significant difference in saturation of blood oxygen and the rate of chylothorax after operation between two groups (P＞0.05).The symptoms and signs including cyanosis and breath were markedly alleviative in all patients.ConclusionBidirectional Glenn shunt without ECC is an effective and safe method for complicated CHD.
ABSTRACT
A 13-year-old girl with asplenia syndrome who previously had undergone left subclavian-to-pulmonary artery shunt after removal of a cavopulmonary shunt with interposition of a short segment of the left superior vena cava was admitted for congestive heart failure. Angiography revealed aneurysmal dilatation of the left superior vena cava. Percutaneous coil embolization of the shunt was successfully performed and the venous aneurysm was diminished. Interposition of a venous component in systemic-to-pulmonary artery shunt should be avoided even after removing a cavopulmonary shunt.
ABSTRACT
Recently, modified Fontan operation is being used for asplenia syndrome. We reviewed 24 patients with asplenia syndrome who underwent surgical intervention. Eleven (45.8%) of them had total anomalous pulmonary venous connection (TAPVC) and 6 of these were accompanied by pulmonary venous obstruction (PVO). Surgical results were poor in the PVO group. In 4 cases with open heart palliation, that is atrium-common pulmonary venous chamber anastomosis, there were 2 operative deaths, 1 late death, and 1 survival. In 18 cases without PVO, statistical analysis (Fisher's exact probability) showed that pulmonary atresia (PA) was a definite risk factor for Fontan candidates (<i>p</i><0.05). In 9 cases with pulmonary stenosis (PS group), there were 5 candidates for the Fontan type operation. In the other 9 cases with PA (PA group) there were no candidates for the Fontan type operation. Only the size and the morphology of the pulmonary artery were significant factors (<i>p</i><0.05) for candidates of the Fontan type operation among the risk factors such as size and morphology of the pulmonary artery, pulmonary vascular resistance and pressure, atrioventricular valve regurgitation, and single ventricular function. Finally, in these 24 cases, there were only 5 candidates (20.8%) for a Fontan type operation. In conclusion, in order to increase candidates for Fontan precedures, it is important to maintain an adequate pulmonary blood flow. Earlier PDA division and pulmonary arteries plasty are the most importantin PA group. In both groups pulsatile bidirectional cavopulmonary shunts may be useful to increase effective pulmonary blood flow without ventricular volume overload, which leads to atrioventricular valve regurgitation.