An uncommon echocardiographic marker for anomalous origin of the left coronary artery from the pulmonary artery: visualization of intercoronary collaterals within the ventricular septum

A rare and striking echocardiographic finding with color-flow Doppler—visualization of intercoronary collaterals within the ventricular septum—in an asymptomatic 5-year-old girl is reported. It strongly suggests anomalous origin of the left coronary artery from the pulmonary artery. Several additional echocardiographic features can secure the diagnosis and allow one to proceed with surgical correction without confirmatory cardiac catheterization and angiocardiography. The ages of our patient and of the seven cases reported previously suggest that these collaterals will more likely be identified beyond infancy and in relatively asymptomatic patients with this congenital anomaly.     

Mitral valve replacement for severe mitral regurgitation in infants with anomalous left coronary artery from the pulmonary artery

Infants with anomalous left coronary artery from the pulmonary artery (ALCAPA) may present with heart failure, mitral regurgitation, and dilated cardiomyopathy. Reestablishment of a two coronary artery system markedly improves the morbidity and mortality. However, the mitral regurgitation may continue to deteriorate despite surgical correction of the ALCAPA because of previous ischemic damage to the papillary muscles and chordae. Surgical intervention, including mitral valve replacement, may be required even during infancy. We present two infants with ALCAPA who required early mitral valve replacement for severe mitral regurgitation and have done well subsequently.     

Circulatory System in Children with Localized Scleroderma

The circulatory system was studied in 43 children (ages 3–18 years, mean 10.6 years; 32 girls, 11 boys) suffering from localized cutaneous forms of scleroderma. The following studies were undertaken: general pediatric examination, cardiologic examination including routine electrocardiogram (ECG), 24-h Holter ECG monitoring, echocardiography plus Doppler study, and a treadmill exercise test. Three children found to have congenital heart malformations were excluded from the study. Children with localized scleroderma were often lighter and shorter than their appropriate controls. The most common ECG abnormality was incomplete right bundle branch block, but other ECG and Holter abnormalities were found as well. Abnormal echocardiographic results were obtained in 16 cases. The abnormalities concerned valvar function (in all heart valves but predominantly in the mitral valve). It was found that the children suffering from scleroderma had different indices than controls for left ventricular mass index, mitral valve function and left ventricular filling. During the exercise test (conducted according to Bruce's protocol) we found no difference between patients and controls.     

Cost-Effectiveness Analysis of Stents,Balloon Angioplasty,and Surgery for the Treatment of Branch Pulmonary Artery Stenosis

Branch pulmonary artery stenosis is a common problem in pediatric cardiology. Treatment has included surgery, balloon angioplasty, and balloon expandable stent placement. It was the purpose of this investigation to demonstrate the cost-effectiveness of each of these modes of treatment. From 1983 to 1994 there were 30 patients admitted for treatment of branch pulmonary artery stenosis only. Data included age at procedure, sex, primary diagnosis, acute and intermediate term success, and complications. Acute success was defined by results at the end of the procedure where intermediate term (IT) success was defined by results at follow-up. Success of a procedure was defined by at least one of the following: an increase in vessel diameter by ≥50% of predilation diameter, a decrease in right ventricular to left ventricular or aortic systolic pressure ratio by ≥20%, or a decrease in peak to peak pressure gradient by ≥50%. The procedure was considered a failure if the previously mentioned criteria were not met or if the patient required a second procedure for the same stenosis. The expense of the procedure (estimated by using the patient charges) were collected from the time of the procedure until December 1994. Because of differing lengths of follow-up, the patients were analyzed separately for procedures and outpatient charges. The total charges were corrected to 1994 dollars using the Medical Consumer Price Index. Thirty patients had 46 separate procedures (12 patients had >1 procedure and 3 had >2 procedures). There were 13 surgeries, 13 balloon angioplasties, and 20 stents. Stents were the most successful (90% acute and 85% IT), but were not statistically superior to surgery (62% acute and IT). Balloon angioplasty was significantly less successful as compared with stents (31% acute and 23% IT), and was not statistically different from surgery over the acute and intermediate term. The charge data showed balloon angioplasty was the least expensive followed by stents and then by surgery. The average total charges per procedure, including outpatient charges, were: surgery $58,068 ± $4372 (standard error), balloon $21,893 ± $5019, stents $33,809 ± $3533 (p < 0.001); excluding outpatient charges: surgery $52,989 ± $3649, balloon $15,653 ± $1691, and stents $29,531 ± $2241 (p < 0.001). Average total charges per patient, including all procedure types and grouped by initial procedure, were: surgery $53,707 ± $6388, balloon $50,040 ± $8412, and stent $34,346 ± $3488 (p= 0.047). Stents were at least as effective as surgery and were more effective than balloon angioplasty in both acute and intermediate term follow-up. Balloon angioplasty was least expensive per procedure but was also least effective. Therefore, intravascular balloon expandable stents are the most cost-effective means available in the treatment of branch pulmonary artery stenosis.     

Anomalous Origin of the Left Coronary Artery from the Pulmonary Artery in Children: 15 Years Experience

This study aimed to illustrate the experience of treating children with anomalous origin of the left coronary artery from the pulmonary artery (ALCAPA). The clinical data for 19 children with ALCAPA admitted to Beijing Anzhen Hospital from August 1993 to June 2009 were reviewed. According to the data, 47.4% (9/19) of the patients had a misdiagnosis of endocardial fibroelastosis, and 15.8% (3/19) had a misdisgnosis of dilated cardiomyopathy. Electrocardiography showed abnormal Q waves with T wave inversion in leads I, avL, and V4-V6 of 18 patients, especially in lead avL. The ratio of proximal right coronary artery diameter to aotic root diameter exceeded 0.20 for 15 of 16 patients. Apical ventricular aneurysm or aneurysmal dilation (52.6%,10/19), enhanced echogenicity of papillary muscles (84.2%, 16/19), and increased coronary collaterals (78.9%, 15/19) were detected frequently during echocardiography. A total of 18 patients underwent cardiac surgery including left coronary artery (LCA) ligation for 1 patient (5.6%), LCA ligation plus coronary artery bypass grafting for 1 patient (5.6%), Takeuchi operation for 7 patients (38.9%), and LCA reimplantation for 9 patients (50.0%). Five patients died in the hospital, and the remainder were asymptomatic during a follow-up period of 6 to 166 months. Their abnormal Q waves gradually regressed, and left ventricular systolic function and size returned to normal with alleviation of mitral insufficiency. The clinical features of ALCAPA are helpful for determining an accurate diagnosis. This anomaly can be treated successfully by several types of operations with good prognosis.     

Serial Pulsed Doppler Assessment of Pulmonary Artery Pressure in Very Low Birth-Weight Infants

We assessed pulmonary artery pressure (PAP) during the early neonatal period in very low birth-weight (VLBW) infants using serial echocardiographic measurements of the ratio of the pulmonary artery acceleration time to the right ventricular ejection time corrected by heart rate [AT:RVET(c)]. Eighty-four VLBW infants weighing less than 1500 g were examined using serial color Doppler echocardiography from 3 hours to day 7 after birth. The AT:RVET(c) of infants born after 30 weeks of gestation showed a rapid, significant increase during the early neonatal period, whereas those of the groups born at less than 30 weeks showed no significant increase before day 14. At 24 hours after birth, the AT:RVET(c) values of VLBW infants did not correlate well with either the ratio of the right preejection period to the right ventricular ejection time on M-mode echocardiography or the pressure gradient between the right ventricle and the right atrium, as estimated by tricuspid regurgitation on pulsed Doppler echocardiography. The AT:RVET(c) value for the chronic lung disease (CLD) group did not differ significantly from that for the oxygen-dependent group at any assessment point. During the early neonatal period, the AT:RVET(c) of VLBW infants, as calculated by pulsed Doppler echocardiography, differed with their gestational age and did not appear to correlate well with PAP. Our data also suggest that AT:RVET(c) values may not be a good predictor of the subsequent occurrence of CLD in VLBW infants.     

Atypical Bland–White–Garland Syndrome with Stenosis of the Origin of the Left Coronary Artery: Catheter Intervention After Mammary Artery Bypass Stenosis and Residual Fistula to the Pulmonary Trunk

A 16-year-old boy with anomalous origin of the left coronary artery from the pulmonary artery, Bland–White–Garland syndrome, underwent a mammary artery bypass grafting to the left coronary artery (LCA) together with closure of the stenosed origin of the left coronary artery. A residual LCA to pulmonary artery fistula and stenosis of the shunt at the implantation site developed that resulted in a dominant perfusion of the LCA from the right coronary artery. Interventional catheterization was performed with stenting of the LCA mammary artery anastomosis and subsequent coil occlusion of the residual fistula. After this intervention the LCA was exclusively perfused by the mammarian bypass with no residual leak to the pulmonary artery.     

Doppler Echocardiography During Exercise to Predict Residual Narrowing of the Aorta After Coarctation Resection

Blood pressure measurements and Doppler echocardiography at rest and during exercise were performed in 36 patients at an average 17 years after coarctation resection. Interest focused on the study of Doppler systolic and diastolic gradients and half-times during exercise. Blood pressure gradients and Doppler values were compared with the degree of narrowing at the site of the anastomosis measured by magnetic resonance imaging. A systolic half-time of >110 ms together with a diastolic gradient of ≥17 mmHg on exercise Doppler sonography predicted a residual isthmic narrowing of >30%. We propose the use of this noninvasive method for identifying patients with narrowing of >30% at the site of the anastomosis after coarctation resection. In these cases evaluation by an imaging technique is necessary to confirm the need for surgery.     

Automated cardiac auscultation for detection of pathologic heart murmurs

Experienced cardiologists can usually recognize pathologic heart murmurs with high sensitivity and specificity, although nonspecialists with less clinical experience may have more difficulty. Harsh, pansystolic murmurs of intensity grade ≥3 at the left upper sternal border (LUSB) are likely to be associated with pathology. In this study, we designed a system for automatically detecting systolic murmurs due to a variety of conditions and examined the correlation between relative murmur intensity and likelihood of pathology. Cardiac auscultatory examinations of 194 children and young adults were recorded, digitized, and stored along with corresponding echocardiographic diagnoses, and automated spectral analysis using continuous wavelet transforms was performed. Patients without heart disease and either no murmur or an innocent murmur (n= 95) were compared to patients with a variety of cardiac diagnoses and a pathologic systolic murmur present at the LUSB (n= 99). The sensitivity and specificity of the automated system for detecting pathologic murmurs with intensity grade ≥2 were both 96%, and for grade ≥3 murmurs they were 100%. Automated cardiac auscultation and interpretation may be useful as a diagnostic aid to support clinical decision making.     

左冠状动脉异常起源于肺动脉22例诊断分析

目的 探讨左冠状动脉异常起源于肺动脉的临床诊断方法.方法 收集本院2000年2月至2007年2月左冠状动脉异常起源于肺动脉患儿22例,分为婴儿组(年龄≤1岁,n=10)和少儿组(年龄＞1岁且≤15岁,n=12),回顾性分析临床表现、心电图和经胸超声心动图表现.其中心电图主要分析如下特征:(1)Ⅰ导联Q波和T波倒置,(2)aVL导联异常Q波和T波倒置,(3)V5-6导联Q波,(4)v4-6导联T波倒置或ST段变化,(5)左心室肥大.经胸超声心动图主要分析如下特点:(1)左冠状动脉与肺动脉的连接,(2)肺动脉内逆向的多普勒血流,(3)左心室乳头肌的回声强度,(4)右冠状动脉扩张,(5)室壁和室隔间侧支循环信号.结果 胸片示心影增大(18/22)、心电图示aVL导联异常Q波和T波倒置(17/22)、超声心动图示汇入肺动脉的逆向多普勒血流(20/22)、前外侧乳头肌强回声(17/22)和侧支循环信号(16/22)在两组患儿中的出现率均很高,差异无统计学意义(P＞0.05);婴儿组的临床症状和心电图示V5-6导联Q波、Ⅰ Q波和T波倒置的出现率均显著高于少儿组(P＜0.05);婴儿组右冠状动脉扩张的出现率要显著低于少儿组(p＜0.05).结论 婴儿和少儿患者的诊断特点不完全相同,胸部X线片示心影增大、心电图示aVL导联异常Q波和T波倒置对本病诊断有重要的提示作用,而超声心动图示逆向汇入肺动脉的彩色多普勒血流、前外侧乳头肌强回声和侧支循环信号等间接征象强烈提示本病的诊断,结合临床病史、心电图和超声心动图检查可以确诊本病.     

The Use of Papaverine in Arterial Sheaths to Prevent Loss of Femoral Artery Pulse in Pediatric Cardiac Catheterization

This study evaluated the efficacy of intraarterial papaverine infusion after pediatric cardiac catheterization in reducing the incidence of lost pulse. This study was conducted at a teaching pediatric hospital and was designed as a randomized, prospective, double-blind, placebo-controlled study. Patients were children 5 years old or less undergoing antegrade and retrograde heart catheterization. The intervention used was an intraarterial infusion of placebo or 1.5 mg/kg papaverine after catheterization. Bilateral femoral artery ultrasound study was performed pre- and postcatheterization, including two-dimensional, color, spectral, and pulsatility index analysis. Outcome measures included clinical loss of pulse, ultrasound findings, plus other clinical factors. Fifty-six patients participated in the study (28 per group). Six patients clinically lost the pulse in the catheterized arteries [four in the papaverine and two in the placebo groups (p > 0.64)]. Clinical loss of pulse correlated with diminished pulsatility index (p < 0.035). The use of papaverine did not prevent decreased pulse or pulsatility index (p > 0.79). Increased arterial time was the only clinical factor associated with lost pulse (p < 0.0004). Pulsatility index <3.34 correlated with loss of pulse in the catheterized artery. The results indicated that intraarterial papaverine infusion after femoral artery catheterization does not prevent loss of pulse in the catheterized artery, and decreased pulsatility index correlates with diminished pulse and represents a good noninvasive method of evaluating femoral artery patency and flow.     

成人型与婴儿型左冠状动脉异常起源于肺动脉的超声诊断

目的总结不同类型左冠状动脉异常起源于肺动脉（ALCAPA）的超声心动图表现,提高对本病的诊断及鉴别诊断能力。方法回顾性分析首都医科大学附属北京安贞医院儿童心血管病中心收治并经手术证实的24例ALCAPA患儿的超声心动图结果,根据侧支循环的发育情况分为婴儿型和成人型,并总结婴儿型和成人型ALCAPA的超声心动图特征。 结果婴儿型和成人型ALCAPA相同的超声心动图特征：①左冠状动脉窦内无左冠状动脉主干开口,其主干直接与肺动脉连接;②左心室高度扩张,室间隔和左室前壁节段性运动障碍,左室收缩功能减低;③左心室广泛纤维化,以心内膜下区域最为显著;④二尖瓣腱索、乳头肌纤维化,回声显著增强;⑤右冠状动脉起源位置正常,内径增宽;⑥左冠状动脉前降支和回旋支血流为逆向灌注（向心性）;⑦收缩期二尖瓣口可见少至大量反流信号。婴儿型和成人型ALCAPA不同的超声心动图表现：彩色多普勒显示成人型侧支循环血流丰富,婴儿型侧支循环少。 结论婴儿型和成人型ALCAPA具有其特异性的超声心动图特征,结合其临床及心电图等表现,可以对本病做出正确诊断。     

Aberrant Left Coronary Artery Arising from the Right Sinus of Valsalva with a Right Coronary Arteriovenous Malformation

An 11-year-old boy presented with myocardial ischemia and was found to have an aberrant left main coronary artery from the right sinus of Valsalva coursing between the aorta and pulmonary artery, as well as a small arteriovenous malformation from a right atrial branch of the right coronary artery to the right atrium. Distinctive echocardiographic findings were supported by angiographic and magnetic resonance imaging studies. Treadmill and scintigraphic stress testing were normal. Corrective surgery was accomplished by a modified technique to unroof the intramural proximal course of the left coronary artery, without postoperative complications. The anatomy and pathophysiology of this rare coronary lesion are reviewed.     

Major coronary anomalies in childhood

Major coronary artery anomalies are extremely rare in childhood. We wanted to assess the historical and diagnostic features and the therapeutic options of three distinct types of coronary artery anomalies: abnormal origin of the left coronary artery from the pulmonary artery (ALCAPA), coronary fistula and coronary stenosis. In a retrospective study, 33 children with these types of coronary artery disease were identified, 15 with ALCAPA, 12 with fistula and six with coronary stenosis. History, physical examination, ECG, X-ray, echocardiography, angiography, therapy and outcome were reviewed. ALCAPA showed distinct typical echocardiographic characteristics. Coronary artery fistula could be identified by a typical murmur and echocardiographic evidence of coronary dilatations. Coronary stenosis should be suspected by the clinical picture in a specific context. CONCLUSION: rare coronary artery anomalies can be accurately diagnosed in childhood. Timely therapy yields good prognosis.     

左冠状动脉起源于肺动脉37例患儿各年龄段的临床特点及诊治

目的分析各年龄段左冠状动脉起源于肺动脉(ALCAPA)患儿的临床特点和诊治情况,提高对此病的认识,减少误诊,提高救治率。方法 1999年4月-2011年3月本院共收治37例ALCAPA患儿。男21例,女16例;年龄(5.0±4.8)岁。其中婴儿组(≤1岁)8例,幼儿组(>1～3岁)10例,大年龄组(>3岁)19例。37例中并二尖瓣关闭不全31例,室壁瘤2例,法洛四联症/ASD、重度肺动脉瓣狭窄/部分型肺静脉异位引流、ASD各1例。对各年龄段临床特点和诊治结果进行回顾性分析。随访结果依据门诊复查结果及电话随访。结果婴幼儿组发病年龄均早于大年龄组;ECG出现Q波及ST-T改变13例(35.1%,13/37例),其中婴儿组5例(62.5%)、幼儿组3例(30.0%)、大年龄组5例(26.3%),3组差异无统计学意义;胸部X片示心脏均明显增大,心胸比婴儿组大于幼儿组和大年龄组,幼儿组大于大年龄组;超声心动图示左心室舒张末径(LVED)3组均增大,以婴儿组增大最明显;左心室射血分数(LVEF)<50%者婴儿组多于幼儿组和大年龄组,幼儿组与大年龄组无明显差异。首次诊断时误诊或漏诊18例,占48.6%(18/37例)。婴儿组1例由于核素扫描提示存活心肌很少,等待心脏移植,另2例家长放弃手术。34例患儿行冠状动脉移植术23例,肺动脉内隧道术8例,冠状动脉搭桥2例,单纯结扎冠状动脉左主干1例。无手术早期死亡。术后有近期随访资料26例(76.5%,26/34例),随访6～130(13.6±18.4)个月,晚期死亡1例。患儿术后LVED均较术前缩小,仍有二尖瓣轻度关闭不全8例。有随访资料患儿中,8例术前LVEF<50%,术后3～6个月LVEF均恢复正常。结论 ALCAPA婴儿患儿临床表现较幼儿及年长儿患儿重。小儿ALCAPA由于缺乏特征性表现,各年龄段均易误诊。对于小儿各年龄段表现为心脏扩大、心功能不全、二尖瓣关闭不全等患儿,要注意除外小儿ALCAPA。可以结合其ECG表现,反复超声ECG探查冠状动脉,必要时加行多层螺旋CT或心血管造影以明确诊断,确诊后应尽快手术治疗。     

Surgical Cutdown of the Right Carotid Artery for Aortic Balloon Valvuloplasty in Infancy: Midterm Follow-Up

A vascular approach for balloon aortic dilatation in infants is of major concern due to the high risk of femoral artery injury. To overcome this problem, a surgical right carotid artery (RCA) approach has been advocated. No reports are available on RCA after neonatal cutdown. Since 1990, RCA cutdown has been performed in 29 infants with congenital aortic stenosis. Periprocedural complications included one asymptomatic occlusion and one transient trauma of the stellate ganglion. At a mean follow-up of 4.4 ± 2.2 years from aortic valvuloplasty, ultrasound assessment of morphology and flow of RCA was performed in 17 children. RCA was patent in all patients. The mean ratio of right/left carotid artery diameter was 0.95 ± 0.16 (range 0.65–1.2). The site of surgical incision could be identified in 5 children in the absence of flow disturbance. A 6-year-old asymptomatic girl, who underwent two procedures, had a 35% reduction of RCA diameter at the site of cannulation and turbulent flow at Doppler interrogation, indicating mild obstruction. Our data demonstrate that RCA is well preserved after neonatal surgical cutdown; asymptomatic obstruction can occasionally be present.     

The Role of Ductal Constriction in Transient Stenosis of the Left Pulmonary Artery

To confirm the hypothesis that narrowing of the left pulmonary artery is produced by ductal closure, Doppler velocities in the pulmonary arteries from 20 premature infants with a patent duct were compared to velocities from 20 with a closed duct. In infants with a patent duct, the mean Doppler velocity in the main, right, and left pulmonary arteries were, respectively, 86 cm/sec ± 5 standard error (SE), 120 ± 7, and 125 ± 8. With closed duct, the comparable velocities were 102 cm/sec ± 7, 129 ± 8, and 190 ± 11. The only significant difference was the higher velocity in the left pulmonary artery associated with a closed duct (p= 0.00003). This physiologic origin of left-branch stenosis associated with ductal constriction should be considered when a murmur is detected after administration of indomethacin (in addition to possible persistent ductal flow), and as an explanation for some murmurs in the normal newborn. No anatomic or physiologic support was found for the theories that murmurs in the neonate were due to ductal flow, or to acute angulation of the branch pulmonary arteries.     

Right Coronary Artery to Left Ventricular Fistula Associated with Left Ventricular Aneurysm

An infant with right coronary artery to left ventricular fistula associated with left ventricular aneurysm successfully underwent a division of fistula under cardiopulmonary bypass. Follow-up angiogram showed the patency of the right coronary artery, no recurrence of fistula, and the regression of the left ventricular aneurysm. Because the lesion may progress with age, early diagnosis and surgical intervention are indicated to prevent later complications.     

Noninvasive Prediction of Pulmonary Artery Pressure in Patients with Isolated Ventricular Septal Defect

Management of patients with isolated ventricular septal defect (VSD) requires information regarding pulmonary artery pressure (PAP). The purpose of this study was to evaluate the individual predictive value of noninvasive methods for assessment of PAP and to determine if any combination of techniques significantly improved their predictive power. We reviewed the clinical history, electrocardiogram, and echocardiogram of 31 patients (age 1.9 ± 1.73 years) who underwent catheterization for isolated VSD. Noninvasive data were compared for patients with mean PAP <20 mmHg (group 1) and those with mean PAP ≥20 (group 2) at catheterization. Fourteen (45%) patients were in group 1 and 17 (55%) in group 2. Doppler estimation of VSD gradient, right ventricular hypertrophy by echocardiogram, interventricular septal orientation, and VSD size had predictive value for elevated mean PAP (p < 0.01). All patients (n= 6) with normal findings in all four variables had normal PAP. All patients (n= 12) with at least three of four abnormal findings had elevated PAP. Six patients in group 1 had at least one variable that incorrectly predicted high PAP, whereas 3 patients with normal findings on three of the four variables nevertheless had elevated PAP. No single noninvasive variable accurately predicted PAP in all cases. However, normal findings for all four significant variables did predict normal PAP and suggest that cardiac catheterization is unnecessary in that setting. However, any other combination of normal and abnormal findings for the four significant variables did not reliably predict PAP and such patients may require catheterization to directly measure PAP.     

Effects of Age and Respiration on Right Ventricular Diastolic Filling Patterns in Normal Children

Right ventricular diastolic filling was assessed in 88 normal Chinese children using Doppler echocardiographic interrogation of diastolic transtricuspid valve flow. Any influences of age and respiration on right ventricular diastolic filling were also investigated. The results showed that age-dependent differences were apparent for filling times, associated with the decrease in heart rate with increasing age. In addition, peak transtricuspid flow velocities were markedly greater in inspiration than in expiration, with corresponding increases in filling times and velocity–time integrals. The influence of age and respiration on transtricuspid flow parameters should be considered in the evaluation of right ventricular diastolic function by Doppler echocardiography. We believe that measurements should be standardized for respiratory phase and that right ventricular diastolic function parameters should be taken at end-inspiration and end-expiration, respectively.