Cardiac arrest in patient with significant pulmonary regurgitation after surgical valvulotomy at 10 years of age for isolated pulmonary stenosis

The number of adult patients with a congenital heart defect has increased. They are 2–3 times more numerous than children suffering from congenital heart defects, therefore, it is important to be aware of the most frequent congenital heart defects in adulthood – atrial septal defect, ventricular septal defect, aortic coarctation, tetralogy of Fallot and pulmonary stenosis. These patients either underwent one or more operations in childhood, or were not operated at all (defect was not significant or inoperable), or the heart defect was not diagnosed (mostly atrial septal defect). Some of the patients for various periods of time stop attending regular follow-ups and being asymptomatic (even in cases of hemodynamically significant defects), they do not seek medical attention. We present the case of a 46-year-old man with gradually progressing pulmonary regurgitation after surgical valvulotomy at 10 years of age. Despite regular follow-ups by the cardiologist, the patient was never referred to a specialized centre and cardiac arrest caused by ventricular fibrillation occurred after physical exercise.     

Hemodynamic studies after total correction of tetralogy of Fallot

Twenty patients were studied by right heart catheterization 5 to 23 months after complete surgical correction of tetralogy of Fallot. The ventricular septal defects had been closed with a polyvinyl sponge and a Teflon prosthesis had been used to widen the right ventricular outflow tract in each case.

Residual systolic gradients between the right ventricle and pulmonary artery were small. Fifteen patients had gradients of 15 mm. or less, and the highest found was 34 mm. Arterial oxygen saturation was normal at rest or with exercise, or both, in nearly all cases. Two patients had definite evidence of a persistent ventricular septal defect with a left-to-right shunt.

Pulmonic regurgitation was present in 13 patients. Despite elevated right ventricular end diastolic and right atrial pressures in some patients, clinical evidence of heart failure was absent in all at the time of catheterization.

A comparison was made of the right ventricular and pulmonary arterial pressure measurements obtained in the operating room upon completion of the cardiac repair with those made at catheterization. It was found that pressure measurements at surgery give a reliable indication of the effectiveness of relief of the right ventricular outflow stenosis.

The operation employed in these patients has relieved the physiologic abnormalities of tetralogy of Fallot. Pulmonic regurgitation is present in many of the patients but is tolerated well at present. The long-term effects of this new abnormality will become clear only after extended observation.     

Accuracy of electrical impedance cardiography for measuring cardiac output in children with congenital heart defects

This study determined whether noninvasive electrical impedance cardiography accurately measures systemic blood flow (cardiac output) in children with congenital heart defects. A total of 37 patients ranging in age from 2 to 171 months underwent complete right- and left-sided heart catheterizations that included simultaneous Fick and impedance measurement of cardiac output. Based on the diagnosis, 4 groups were formed consisting of a control group (n = 11) with no shunts, a group with intracardiac left-to-right shunting and an atrial septal defect (n = 7), another with a ventricular septal defect (n = 12) and an extracardiac left-to-right shunting with patent ductus arteriosus group (n = 7). Impedance values for systemic blood flow were compared with systemic and pulmonary blood flow obtained by the direct Fick method with measured oxygen consumption. The difference between impedance and Fick systemic blood flow was less than or equal to 5% in each of the 4 groups. The highest correlation between impedance and Fick systemic blood flow was with the atrial septal defect group (r = 0.89) and lowest with the ventricular septal defect and control (r = 0.69) groups. Fick pulmonary blood flow was significantly greater than impedance or Fick systemic flow in all 3 shunt groups. Impedance cardiography accurately measured systemic blood flow in children without shunts or valvular insufficiency. Likewise, systemic blood flow was accurately measured by impedance in the presence of intracardiac left-to-right shunts (atrial and ventricular septal defects) and extracardiac left-to-right shunts (patent ductus arteriosus).(ABSTRACT TRUNCATED AT 250 WORDS)     

Extravascular lung water in children immediately after operative closure of either isolated atrial septal defect or ventricular septal defect

Extravascular lung water (EVLW) was measured in 16 patients with congenital heart disease by the cold green dye, double indicator dilution technique. Five patients with optimally corrected tetralogy of Fallot served as controls, and EVLW in this group was 4.7 +/- 0.5 ml/kg (111 +/- 13 ml/m2) (mean +/- standard deviation). In 5 asymptomatic patients with atrial septal defect (ASD), normal pulmonary artery (PA) pressure and increased pulmonary blood flow, EVLW was 5.7 +/- 2.8 ml/kg (132 +/- 63 ml/m2), which was not significantly different from the value of control patients. However, in 6 patients with ventricular septal defect, PA hypertension, normal left atrial pressure and an equivalent left-to-right shunt to ASD patients, EVLW was 15.9 +/- 3.8 ml/kg (270 +/- 60 ml/m2). This was significantly different from values in both control and ASD patients (p less than 0.01). It is concluded that in the face of normal pulmonary vascular resistance, PA pressure is transmitted to the microvasculature, causing hydrostatic pulmonary edema. Other factors that may be implicated in the pathogenesis of pulmonary edema, such as increased pulmonary blood flow and relative lymphatic insufficiency in infants, cannot be excluded.     

Changes in ventricular size and plasma renin activity after cardiac surgery in children.

Plasma renin activity and one-dimensional echocardiographic measurements of the left atrium and left ventricle as well as function indices were studied repeatedly in 20 children with various cardiopathies (ages: 9 months to 15 years) before and after corrective surgery. Nine children had tetralogy of Fallot, four had pulmonary stenosis, four had rheumatic heart disease, two had ventricular septal defect, and one had atrial septal defect. Plasma renin activity was normal preoperatively, but increased significantly immediately after surgery, was still significantly higher on the 12th postoperative day and returned to normal six to eight weeks after surgery. Patients with tetralogy of Fallot and pulmonary stenosis had higher plasma renin activity values than the others. There was a positive correlation between plasma renin activity and postoperative percentage change of the left ventricular dimension. In patients with tetralogy of Fallot and pulmonary stenosis, this meant that plasma renin activity became normal when the preoperatively small left ventricle reached its normal dimension. This adjustment occurred slowly over a period of two months. In rheumatic heart disease and left-to-right shunt lesions, plasma renin activities became normal when the preoperatively dilated left ventricle decreased in size towards normal values; the plasma renin activities of these patients had reached normal levels by the fifth postoperative day. The renin secretion is modulated by various factors: of these, ventricular size and pulmonary venous return seem to be of importance.     

Current role of radionuclide imaging in pediatric cardiology

Three main nuclear medicine methods are used in paediatric cardiology: sequential first-pass radionuclide imaging of the cardiac cavities, radionuclide equilibrium ventriculography and radionuclide myocardial imaging. Valuable functional information is obtained, and invasive explorations can be avoided in an ever increasing number of cases. Of particular interest is left-to-right shunt measurement which indicates that atrial septal defects must be surgically corrected when the pulmonary/systemic flows ratio (QP/QS) is above 2. This technique is also useful to evaluate the tightness of repairs in ventricular and atrial septal defects. Radionuclide studies of the right and left ventricles may detect dysfunction in one or the other cavity. The left ventricular ejection fraction is reduced in myocardiopathy an in aortic or mitral valve diseases seen at a late stage. The right ventricular function is often abnormal, notably during exercise, after repair of the tetralogy of Fallot and after atrial correction of complete transposition of the great arteries. An altered ejection fraction in patients with single ventricle is also a sign of deterioration. Right ventricular diastolic overload evaluated by radionuclide equilibrium ventriculography correlates with the QP/QS ratio value in atrial septal defects and with the inducibility of ventricular tachycardia by endocavitary pacing in repaired tetralogy of Fallot. Thallium 201 myocardial imaging provides information on myocardial ischaemia, notably that associated with congenital abnormalities of the coronary arteries. Its use had now been extended, albeit with some limitations, to the evaluation of right ventricular systolic overload. Other radionuclide techniques are being developed with new tracers: Kryton 81m for studies of the right ventricle, short-lived radionuclides for first-pass studies, Iodine 123-labelled fatty acids for myocardial imaging. More recently, some substrates, such as deoxyglucose, have been labelled with positron emitters permitting in vivo metabolic studies.     

Comparison of different approaches for pediatric congenital heart diseases

To compare the clinical results of different surgical approaches for congenital heart disease in pediatric patients, 1669 cases of atrial septal defect, ventricular septal defect, or tetralogy of Fallot, which were corrected from January 1999 to December 2001, were classified according to approach (sternotomy, ministernotomy, or minithoracotomy). In cases of ventricular septal defect, the incidence of pulmonary complications was significantly higher in the minithoracotomy group than in the full sternotomy or ministernotomy groups. In patients with tetralogy of Fallot, hemoglobin concentration was higher, oxygen saturation was lower, and more patients required a transanular patch in the sternotomy group than in the other groups, but the clinical results were similar. Patients with complex defects or severe pulmonary hypertension should undergo a full sternotomy.     

婴幼儿先天性心脏病的外科手术与围术期处理

目的总结婴幼儿先天性心脏病的外科手术经验。方法自2000年1月至2004年12月共为133例,年龄5￣36个月,体重6￣15kg的先天性心脏病患儿施行外科手术治疗。全组非体外循环动脉导管结扎术29例;体外循环手术104例,包括室间隔缺损、继发孔房间隔缺损、肺动脉瓣狭窄、部分型房室间隔缺损、部分型肺静脉异位引流、法乐四联症、房间隔缺损并右室流出道梗阻和其他复合畸形。结果全组术后住院死亡6例,死亡率4.5%。术后主要并发症包括低心排综合征、呼吸衰竭、心律失常、肺部感染、出血等。死亡原因包括重度低心排综合征、严重室性心律失常、肺动脉高压危象等。结论心外科、心儿科、麻醉科等人员的密切配合,心内、外科镶嵌治疗成为婴幼儿心脏外科手术成功的重要保证。     

Complete atrioventricular septal defect with tetralogy of Fallot: diagnosis and management.

OBJECTIVE--To report recent experience of patients with complete atrioventricular septal defect and tetralogy of Fallot, with emphasis on anatomical features, diagnosis, and management. DESIGN--Case notes were reviewed and patients were assessed at follow up by clinical examination and cross sectional and Doppler echocardiography. SETTING--Tertiary cardiothoracic referral centre. PATIENTS--Between 1987 and 1992 13 patients with atrioventricular septal defect and tetralogy of Fallot (12 with concordant and one with double outlet ventriculoarterial connections) underwent surgery; 10 underwent complete intracardiac repair. 11 patients had Down's syndrome. The complete diagnosis was established preoperatively by cross sectional echocardiography in all but one patient. A tri-leaflet left atrioventricular valve as seen in parasternal short axis views was the diagnostic feature of atrioventricular septal defect, with tetralogy of Fallot diagnosed from the presence of anterocephalad deviation of the outlet septum producing subvalvar pulmonary stenosis as seen in subcostal right anterior oblique views. INTERVENTIONS--Total correction consisted of closure of the atrioventricular septal defect by a combined right atrial and ventricular approach, reconstruction of the atrioventricular valves, and relief of the obstruction within the right ventricular outflow tract. Separate patches were used to close the atrial and ventricular septal defects. Modified Blalock-Taussig shunts were performed in three patients, who await intracardiac repair. Surgical correction was carried out at mean (range) age of 5 (2 to 15) years. MAIN OUTCOME MEASURES--Diagnostic methods, surgical results, and functional state after complete correction. RESULTS--The presence of an atrioventricular septal defect was missed preoperatively in one patient with tetralogy of Fallot. The characteristic goose neck deformity on the left ventriculogram was not present and the tri-leaflet nature of the left atrioventricular valve was not sought on echocardiography. Of the 10 patients who underwent complete repair, nine are alive and one died 34 days after operation with adult respiratory distress syndrome. Examination at necropsy showed an excellent surgical correction. Mean (range) follow up was 23 (8 to 48) months. All nine patients are alive and well (New York Heart Association Class 1). CONCLUSION--Accurate diagnosis and staged management with improved surgical techniques have lowered mortality of this complex combination of cardiac defects. The current policy of this group is to recommend a systemic to pulmonary arterial shunt procedure for symptomatic children younger than 2 years and total correction in older children.     

Surgical experience with congenital heart disease in Down's syndrome

Children with Down's syndrome and congenital heart defects have multiple problems. The role of cardiac surgery in the management of these patients was investigated by reviewing the clinical data, hospital course and follow-up of 21 patients (9 males and 12 females, age range 1 month to 14 years) with Down's syndrome and congenital heart defects operated in our institute. Twelve (57%) of these were infants and nine (43%), older children. Five were in congestive cardiac failure, four were hypothyroid. The heart lesions ranked in incidence as follows: atrioventricular septal defect 7 (33.3%), tetralogy of Fallot 3 (14.3%), tetralogy of Fallot & atrioventricular septal defect both 2 (9.5%), double outlet right ventricle with pulmonary stenosis 1 (4.8%), patent ductus arteriosus 2 (9.5%), patent ductus arteriosus plus coarctation 1 (4.8%), ventricular septal defect 2 (9.5%), atrial septal defect plus ventricular septal defect 1 (4.8%), atrial septal defect plus patent ductus arteriosus plus right pulmonary artery stenosis 1 (4.8%) and transposition of great arteries with multiple ventricular septal defect 1 (4.8%). Four (19%) patients had palliative procedures while the rest (81%) underwent primary repair. All survived the operation. The post-operative period was complicated in 6 (28.5%), with respiratory infections in 3, pulmonary hypertensive crisis in 2 and complete heart block in 1. The early mortality was 0, while there were 2 (9.5%) late deaths. The number of hospitalisations was markedly reduced according to the parents. Follow-up showed near normal pulmonary artery pressure in 50 percent children with large shunts and a good developmental spurt was seen in 60 percent. From a purely surgical viewpoint, the prognosis for children with Down's syndrome and congenital heart disease is good.     

Uncorrected tetralogy of Fallot in an 86-year-old patient

This report describes the presentation and evaluation of an elderly man with uncorrected tetralogy of Fallot. The patient had remained fairly asymptomatic for much of his life. He presented to the hospital at age 86 with new-onset atrial fibrillation with rapid ventricular response and a non-ST-segment elevation myocardial infarction. Transthoracic and transesophageal echocardiography revealed infundibular pulmonic stenosis with a ventricular septal defect, overriding aorta, and right ventricular hypertrophy, findings consistent with unrepaired tetralogy of Fallot. Severe right ventricular pressure overload was also present. Coronary angiography revealed nonobstructive coronary artery disease. It was felt that the rapid atrial fibrillation resulted in right ventricular subendocardial ischemia that improved following restoration of sinus rhythm. After a systematic literature search, the authors believe this case represents the oldest reported patient with the diagnosis of uncorrected tetralogy of Fallot and serves as an example of a well-balanced congenital shunt.     

心内直视下镶嵌治疗肌部室间隔缺损

目的 探讨心内直视下镶嵌治疗肌部室间隔缺损（mVSD）的手术方法及临床疗效.方法 2008年1月至2013年7月,在体外循环心内直视下镶嵌治疗肌部室间隔缺损29例,男19例,女10例,年龄2个月～7岁;其中单个肌部室间隔缺损7例,多发室间隔缺损22例.合并法洛四联症5例,完全性大血管转位1例,肺动脉瓣狭窄3例,房间隔缺损6例,主动脉缩窄3例.合并心脏畸形均同期手术纠治.结果 死亡2例,死亡率6.9％.术后随访3个月～3年,无远期死亡,封堵器边缘少量残余分流3例,无封堵器偏移、二尖瓣反流、主动脉瓣反流、Ⅲ度房室传导阻滞.结论 心内直视下镶嵌治疗肌部室间隔缺损是一种安全、简便、有效的方法.     

A simple method for insertion of a left atrial catheter in congenital heart disease

A relatively simple and safe method is described for the insertion of a left atrial pressure line in patients undergoing operation for repair of congenital heart defects. This method has been utilized successfully in 12 patients undergoing repair of a ventricular septal defect and tetralogy of Fallot.     

Congenital heart disease in Down's syndrome patients: a decade of surgical experience

Patients with Down's syndrome represent a significant subset of patients with congenital heart disease. Fifty-five patients with Down's syndrome have undergone surgical treatment for congenital heart disease at our institution in the past decade. Twenty-six had atrioventricular canal, 11 had ventricular septal defect, 7 had secundum atrial septal defect, 7 had tetralogy of Fallot, 3 had primum atrial septal defect and 1 patient had double outlet right ventricle. The thirty day mortality following operative intervention was 16.4%. Mortality was highest for tetralogy of Fallot followed by atrioventricular canal and ventricular septal defect. Long term mortality for all lesions was 27.3% over our follow-up period which averaged 33 months. Thirty day mortality compared similarly to previous reports of surgically treated Down's syndrome patients. When compared to our patients without Down's syndrome, the Down's population did not exhibit an increased risk for surgical treatment of congenital heart disease.     

Two-dimensional echocardiographic identification of hemitruncus: Anomalous origin of one pulmonary artery from ascending aorta with the other pulmonary artery arising normally from right ventricle

Two-dimensional echocardiography (2DE) was performed on a 2300 gm newborn who presented with congestive heart failure. Clinical examination suggested left-to-right shunt with pulmonary hypertension. Cardiac catheterization and angiography demonstrated origin of the right pulmonary artery (RPA) from the aorta. Retrospectively, this feature (anomalous RPA) had been present on 2DE. A second patient aged 2 years had evidence of ventricular septal defect with large left-to-right shunt early in life. Gradual development of infundibular stenosis and clinical cyanosis ensued. 2DE showed subaortic ventricular septal defect with infundibular stenosis. Cardiac catheterization and angiography confirmed tetralogy of Fallot with origin of the left pulmonary artery (LPA) from the aorta. Retrospective review of 2DE showed the anomalous LPA arising from the side of the ascending aorta.     

Hemodynamic effects of dynamic exercise in children and adolescents with moderate-to-small ventricular septal defects

We studied the hemodynamic effects of dynamic exercise during cardiac catheterization in 35 children and adolescents with small-to-moderate ventricular septal defects. Eighteen of them exercised at 25% and 50% of their maximum workload and 17 exercised at 60%. There was no significant difference between the two groups with respect to age and body mass, height, and surface area. The changes evoked by exercise showed the same pattern at the different workloads, although they were more marked at the higher than at the lower percentage of maximum workload. During exercise the pulmonary vascular resistance did not change, in contrast to the systemic vascular resistance, which decreased. The pulmonary and systemic blood flows both increased, while the left-to-right shunt flow did not change, which led to a decrease of the left-to-right shunt fraction. As the heart rate increased and the shunt flow did not change, the shunt volume per beat decreased during exercise. We conclude that in patients with small-to-moderate ventricular septal defects the hemodynamic effects of dynamic exercise are favorable because the normal rise in systemic blood flow occurs without a corresponding increase in left-to-right shunt flow. Consequently, children and adolescents with such defects should not be restricted in their dynamic exercise activities.     

新鲜自体心包在心脏手术中应用的经验

目的介绍新鲜自体心包在心脏手术中应用的经验。 方法我院在321例心脏手术中应用了未经戊二醛处理的新鲜自体心包作为修复材料,应用范围广泛,包括先天性心脏病、风湿性心脏病和心脏肿瘤。 结果术后早期死亡20例,手术死亡率6.1%。并发症1例法乐四联症根治术后室间隔缺损残余漏,经二次手术修补治愈出院。3例室间隔缺损修补术后少量残余漏,随诊观察。全组75.7%的病人获得随诊,无术后溶血、栓塞、感染性心内膜炎、补片钙化及心包片瘤样膨出等并发症。 结论新鲜自体心包是心脏手术的优良修复材料。     

Transcatheter patch occlusion of perimembranous ventricular septal defects

Sixteen surgical candidates for ventricular septal defect correction were brought to the catheterization laboratory for transcatheter patch occlusion. There were 3 cases of nonrestrictive ventricular septal defects, including 2 with malalignment (tetralogy of Fallot). All patients, except those with tetralogy of Fallot who were cyanotic, had large left-right shunts. They were all corrected through the femoral vein. All defects with the exception of 2 were successfully occluded (12 full occlusions, 2 residual shunts). On follow-up, there were no embolizations, aortic insufficiency, or other complications. The method appears effective and relatively safe, and could challenge the current surgical standard of treatment.     

Etiologic relations among categories of congenital heart malformations.

Pairs of siblings with congenital heart malformations of different types were analyzed for evidence of nonrandom association of defects within families that might suggest a genetic predisposition common to two or more kinds of malformations. An excess of pairs was noted for tetralogy of Fallot and pulmonary stenosis, tetralogy of Fallot and transposition of the great vessels, and tetralogy of Fallot and ventricular septal defect, thus suggesting that there may be a developmental relation between these lesions. This finding is supported by a recent study in the Keeshond dog demonstrating a genetic predisposition common to tetralogy of Fallot, pulmonary stenosis and ventricular septal defect. Thus the method does seem capable of revealing etiologic relations, probably genetic, among different types of cardiac lesions. Data on risks of recurrence for siblings of children with these defects will now have to be refined to take into account the possible recurrence of related lesions.