Acquired coronary artery fistula after open heart surgery for congenital heart disease

BACKGROUND: Most coronary artery fistulas were reported as congenital. Acquired coronary artery fistula occurring after cardiac surgery has rarely been reported. METHODS: From 1998 to 2003, 10 patients with coronary artery fistula detected by echocardiography after open heart surgery for congenital heart disease were included. Their ages ranged from 2 months to 41 years (median 4.2 years). The underlying heart disease was tetralogy of Fallot in five patients, ventricular septal defect in three, double chamber right ventricle in one, and transposition of the great arteries with ventricular septal defect in the remaining one. RESULTS: Of these 10 patients, the coronary artery fistula originated from the left coronary artery in four, right coronary artery in two, and unknown origin in the remaining four. The coronary artery fistula drained into the right ventricle in nine and into the left ventricle in the remaining one. The incidence of acquired coronary artery fistula after open heart surgery for congenital heart disease was 0.44% (8/1832). The identified risk factors for acquired coronary artery fistula were reoperation and right ventricular muscle resection in ventricular septal defect. After follow-up for 0.5-12 years (mean 4.1+/-3.3 years), the coronary artery fistula persisted, but neither symptoms nor significant left-to-right shunt was noted. CONCLUSIONS: Acquired coronary artery fistula is a rare complication after cardiac surgery. Reoperation and resection of right ventricular hypertrophic muscle increase the risk of this complication. Although shunt flow did not increase during follow-up, the significance of acquired coronary artery fistula needs further investigation.     

Prevalence des cardiopathies infantiles symptomatiques au Centre Hospitalier Régional de Louga,Senegal

The management of congenital or acquired infantile heart diseases in sub-Saharan African countries still presents problems, particularly with diagnosis and access to surgical treatment. Our objectives were to describe the heart diseases observed in the paediatric setting of the Louga Regional Hospital (LRH) and report their short-term evolution.In the study period from 1 July 2009 to 31 December 2012, 82 children out of 18 815 presented with heart disease, which was a prevalence of 4.3/1 000. There was a female predominance, with a ratio of 1.2. The most frequent presenting conditions were dyspnoea at 47.5%, followed by heart murmurs at 35.3%, and congestive heart failure at 13.4%. Congenital heart diseases were the most frequent, representing 69.5% of the cases, followed by acquired heart diseases at 29.3%, and mixed-type cases at 1.2%. The most frequently encountered congenital heart diseases were ventricular septal defect (24.4%), followed by atrioventricular septal defect (12.2%), tetralogy of Fallot (9.8%) and patent ductus arteriosus (7.3%). Acquired heart disease was represented by rheumatic heart disease, found in 25.6% of the cases, and tuberculous pericarditis in 3.7%. The mortality rate was high, with 20 children dying (24.4%) during the study period. Only 13 out of 82 patients (15.9%) were operable and surgery was carried out in France, courtesy of the association Humanitarian Mécénat Chirurgie Cardiaque.Infantile heart diseases were therefore not very frequent in the paediatric unit of Louga Regional Hospital. However, congenital heart disease was more frequent than acquired heart disease, with a high mortality rate. Access to surgery remains limited.     

Three-Dimensional Echocardiography of Right Heart Pathology

Three-dimensional (3-D) echocardiography uses sequentially acquired tomography echocardiographic data, which is gated to the cardiac cycle, to reconstruct 3-D views of the heart. So far, this technique has been used primarily to evaluate left-sided heart structures. This report focuses on congenital and acquired right-sided heart pathologies that have been visualized by 3-D echocardiography. In addition to reviewing the literature, several representative figures are included illustrating the unique ability of 3-D echo to elucidate complex right heart anatomy. After a brief introduction to the technical aspects of 3-D echocardiography, the discussion centers on evaluation of congenital heart disease and right-sided masses, determination of right ventricular mass and volume, and evaluation of right-sided valvular heart disease. Congenital heart diseases that are reviewed include atrial septal defect (location, size, efficacy of repair), ventricular septal defect, and congenital heart disease in the fetus being evaluated in utero. Evaluation of right-sided masses, including tumors, vegetations, and thrombi, is reviewed. Methods of determining right ventricular volume and mass using 3-D echo are discussed. Evaluation of valvular heart disease, including Doppler analysis of regurgitant flow, is examined. Finally, special attention is given to the perioperative and intraoperative use of 3-D echocardiography for patients with these conditions. The conclusion summarizes the current and potential future uses of 3-D echocardiography.     

Occurrence and pattern of congenital heart diseases in a rural area of sub-Saharan Africa

Summary

The extent of congenital heart disease in Cameroon remains largely unknown. The aim of this study was to determine the occurrence and pattern of congenital heart diseases in the Cardiac Centre of St Elizabeth Catholic General Hospital, situated in a rural area of Cameroon.

Methods

Between November 2002 and November 2008, a population of 2 123 patients with suspected cardiac pathologies were consulted at St Elizabeth Catholic General Hospital referral cardiac centre. Of these patients, 292 subjects were recruited for the study, based on detection of (1) precordial murmurs and/or cardiomegaly on chest X-ray examination, or (2) congenital heart diseases on transthoracic Doppler echocardiography examination.

Results

Congenital heart diseases and inorganic murmurs were found in 95.5 and 4.5% of the patients, respectively. Congenital heart diseases included tetralogy of Fallot (26.1%), isolated ventricular septal defect (38.8%), atrioventricular cushion defect (7.3%), isolated atrial septal defect (2.8%), arterial duct cases (12.4%), common arterial trunk (1.3%), isolated stenosis of the pulmonary artery (2.6%), coarctation of the aorta (1.1%), congenital mitral valve regurgitation (1.2%), atresia of the triscupid valve (1.6%), double-outlet right ventricle (2.1%), anomalous pulmonary venous return (1.5%) and left isomerism (1.2%).

Conclusion

Our data show that there is a high occurrence of congenital heart disease in this hospital in a rural zone of sub-Saharan Africa and that isolated ventricular septal defect is the predominant pathology. Post-surgical follow up remains very challenging as many parents cannot afford their children’s medical treatment or are generally not well educated.     

新生儿先天性心脏病的超声心动图诊断—附1294例分析

目的和方法：采用彩色多普勒超声心动图检查新生儿先心病1294例,对检出的各类畸形进行分类及疾病顺们的排列,同时对部分有手术或尸解的病例进行结果对照。结果：非青紫型先心病中,室间隔缺损居本组首位,动脉导管未闭及房间隔缺损分别居第二,三位,青紫型先心病中大动脉转位居首位,法洛四联征及肺动脉闭锁分别居第二,三位。57例有手术或病理结果对照超声诊断单一畸形符合率为100％,复杂畸形诊断符合率为91．2％,结论：彩色多普勒超声心动图诊断新生儿先天性心脏病快速,安全,准确性高 ,是新生儿期先天性心脏病早期确诊及分型的有效方法。     

Hypertrophic cardiomyopathy: Recent observations regarding the specificity of three hallmarks of the disease: Asymmetric septal hypertrophy,septal disorganization and systolic anterior motion of the anterior mitral leaflet

Neither asymmetric septal hypertrophy (ASH), marked cell disorganization in the ventricular septum nor systolic anterior motion of the anterior mitral leaflet (SAM) is pathognomonic of hypertrophic Cardiomyopathy. However, each is uncommonly found in patients with other cardiac disorders and is therefore a highly specific hallmark of hypertrophic cardiomyopathy. Disproportionate septal thickening does occur in about 10 percent of older children and adults with various acquired or congenital heart diseases. In these patients it usually appears to be secondary to the underlying hemodynamic state. However, disproportionate septal thickening is the usual finding in the developing embryonic and fetal heart and it is relatively common (prevalence rate about 25 percent) in normal neonates and infants with congenital heart disease.Likewise, although cell disorganization in the ventricular septum may occur with other cardiac malformations, extensive disorganization is present in about 90 percent of patients with hypertrophic cardiomyopathy and in only about 5 percent of patients with other cardiac diseases. Finally, systolic anterior motion of the anterior mitral leaflet is characteristic of those patients with hypertrophic cardiomyopathy who have obstruction to left ventricular outflow, and it rarely appears (prevalence rate about 3 percent) under basal conditions in other hemodynamic states or cardiac diseases. Hence, in analyses comprising over 1,600 patients the specificity of asymmetric septal hypertrophy, marked septal disorganization and systolic anterior motion of the anterior mitral leaflet was at least 90 percent (90, 93 and 97 percent, respectively). Furthermore, the sensitivity of extensive septal disorganization for hypertrophic cardiomyopathy was 90 percent.The data currently available therefore suggest that the vast majority of patients fulfilling the basic anatomic criteria for hypertrophic cardiomyopathy (that is, a hypertrophied nondilated left ventricle in the absence of a cardiac or systemic disease that itself could produce left ventricular hypertrophy) have a distinct disease entity with diverse clinical manifestations. The majority of such patients appear to have a genetically transmitted disease, but it is not known precisely what proportion of these patients have phenotypically similar but etiologically separate disease entities.     

Echokardiographische Diagnostik angeborener Herzfehler im Erwachsenenalter

Echo and Doppler echocardiographic procedures have gained special importance in the diagnostics of congenital diseases in adults. These procedures permit detailed visualization of the pathomorphology of the heart as well as reliable evaluation of the hemodynamic changes. There are differentiated indications for the various procedures, such as transthoracic and transesophageal echocardiography, Doppler and color-Doppler echocardiography, contrast echocardiography and 3-dimensional echocardiography. This article discusses the opposition of the various echo and Doppler echocardiographic procedures with respect to the diagnostics of the most frequent non-operated congenital diseases in adults. The pathomorphology of the various congenital diseases will be summarized and then the important echocardiographic criteria presented which are decisive for the diagnostic procedure. In simple congenital malformation of cardiac valves, such as bicuspid aortic valve (Figure 1: aortic ring abscess), pulmonary valve stenosis (Figure 2), Ebstein's anomaly (Figure 3) or malformations of the mitral valve (Figure 4: cleft in the anterior mitral cusp), the diagnosis can often be made using transthoracic echo and Doppler echocardiography, and the severity of the defect determined. However, the sonographic conditions, especially in adults, are frequently too limited to permit recognition of detailed smaller changes, so that transesophageal examination is required to finally confirm the diagnosis in these patients. In the diagnostics of diseases of the left ventricular outflow tract and the thoracic aorta, such as subvalvular aortic valve stenosis (Figure 5), the sinus of Valsalva aneurysm or the coarctation of the aorta (Figure 6), the left ventricular outflow tract can be evaluated morphologically from a transthoracic procedure and the accelerations of flow can be recorded by continuous wave Doppler. If there is no sclerosis of the fibrous membrane, these can often not be depicted by transthoracic procedures, so that a supplementary transesophageal examination is meaningful. This is required in any case for diseases of the descending thoracic aorta. In the case of congenital lesions, such as atrial septal defects (Figure 7: anomalous pulmonary venous return, Figure 8: 3-dimensional visualization of an atrial septal defect, Figure 9: sinus venosus defect), ventricular septal defect or a patent ductus arteriosus Botalli (Figure 10), color-Doppler and contrast echocardiography have become especially important. Transesophageal examination is also indicated for these congenital diseases for direct depiction of the defect as well as for precise evaluation of the shunt. Moreover, in atrial septal defects, it has been shown that a 3-dimensional echocardiography provides additional advantage with respect to spatial relationship of the defect to the other cardiac structures, as well as presenting dynamic changes during a heart cycle. Extensive knowledge of complex congenital heart disease, such as tetralogy of Fallot (Figure 11), complete transposition of the great arteries, congenitally corrected transposition of the great arteries (Figure 12), the double-outlet right ventricle, truncus arteriosus communis, the cor triatriatum, tricuspid atresia (Figure 13) or the univentricular heart (Figure 14) usually requires performance of a transthoracic echo- and Doppler echocardiographic examination to assess the pathomorphological changes and to examine hemodynamics. In the majority of patients, supplementary transesophageal echocardiography and an echo contrast examination are important. Initial examinations using 3-dimensional echocardiography are very promising in this connection and with respect to the exact spatial presentation of pathoanatomical structures.     

Multimodal imaging in the diagnosis of isolated left ventricular noncompaction

Left ventricular noncompaction (LNVC), first described in 1984, is a rare congenital cardiomyopathy that is thought to be caused by arrest of normal embryogenesis of the endocardium and the myocardium, and characterized by multiple prominent trabeculations with deep intertrabecular recesses. LVNC can be associated with other congenital cardiac abnormalities such as atrial septal defect and ventricular septal defect, but it can occur in isolation. The clinical manifestations of the disease are variable, ranging from no symptoms to signs of heart failure, systemic emboli, and ventricular arrhythmias. The diagnosis is established by two dimensional echocardiography or magnetic resonance imaging. We report the case of a 44-year-old man with LVNC and discuss the role of multimodal imaging in the diagnosis and assessment of the disease.     

Congenital heart malformations associated with dilated cardiomyopathy

From June 1977 to February 1986 we studied 31 children with dilated cardiomyopathy. Seven patients (22%) aged 8 months to 3 years had associated congenital cardiac lesions--tetralogy of Fallot (3), coarctation of the aorta (2), ventricular septal defect (1) and Ebstein's malformation (1). The diagnosis was confirmed by hemodynamic and angiographic studies in 5 patients and by autopsy in 2. Although different congenital lesions were present, all 7 patients had congestive heart failure, flattening of T-waves or ST-segment depression on the electrocardiogram and significant decrease in the left ventricular shortening fraction as judged by echo. We conclude that the incidence of dilated cardiomyopathy associated with congenital heart malformations is high in our pediatric population. The clinical status of patients appears to be more dependent on the improvement of dilated cardiomyopathy and less dependent upon the underlying congenital heart malformations.     

先天性心脏病介入治疗相关的心脏压塞病例分析

目的 分析与先天性心脏病介入相关心脏压塞的原因及处理方法.方法 4例患者,女性3例,男性1例;年龄18～64岁;3例表现为介入术中血压急剧下降、心率增快及意识丧失(1例).1例为术后12 h出现胸闷,血压下降,经床旁超声证实心脏压塞.3例患者为房间隔缺损,1例为室间隔缺损;4例患者均经X线透视及超声心动图检查以明确诊断,并立即行床旁心包穿刺引流,同时辅以扩容、升压及中和肝素处理.结果 4例患者均存活,2例患者行心包引流后,经常规内科保守治疗,循环仍不稳定,急诊行外科手术治疗;1例患者给予输血治疗后,血压稳定,2天后拔除心包引流导管;1例患者停用抗凝药物,引流约300 ml心包积液后,血压稳定,1天后拔除引流导管.结论 心脏压塞是先天性心脏病介入治疗的严重并发症,规范的操作技术能有效避免其发生.     

Impact of 2-Dimensional echocardiography on the management of distressed newborns in whom cardiac disease is suspected

The course and management of 40 consecutive newborns (aged less than 2 weeks) who presented with signs and symptoms of congenital heart disease were reviewed to determine the impact of 2-dimensional (2-D) echocardiography on their subsequent management. Of the 40 patients with congenital heart disease, 60% did not undergo cardiac catheterization. Forty-two percent of the patients who were treated surgically went directly to operation without preoperative cardiac catheterization. Only 40% of the patients with congenital heart disease required cardiac catheterization in the newborn period, and 43% of these procedures were primarily therapeutic (that is, balloon atrial septostomy). In each patient 2-D echocardiography correctly identified the major cardiac malformation and there was good agreement with angiographic, surgical, and autopsy findings. The most commonly overlooked defect was a patent ductus arteriosus. Thus, 2-D echocardiography not only allows diagnosis of congenital heart disease in the newborn but can expedite clinical management. No longer is cardiac catheterization necessarily the primary means for an anatomic diagnosis of congenital cardiac malformations in the newborn.     

Wolff-Parkinson-White syndrome and cardiopathies

Forty-nine cases of Wolff-Parkinson-White syndrome (WPW) were diagnosed out of 10 750 patients with cardiac disease (0.45 p. 100), 24 cases out of 3 761 congenital malformations and 25 cases in the 6 989 patients with acquired heart disease. Right ventricular pre-excitation was recorded in 31 cases; 13 in the lateral zone, 12 in the posterior paraseptal zone and 6 in the anterior paraseptal zone. Left ventricular pre-excitation was recorded in 18 cases: 8 in the lateral zone, 5 in the anterior paraseptal and 5 in the posterior paraseptal zones. WPW and congenital heart disease: Out of 20 cases of Ebstein's anomaly, 5 cases of WPW were observed: 4 right posterior and 1 right lateral pre-excitations. Out of 218 cases of hypertrophic obstructive cardiomyopathy, 7 cases of WPW were observed, 4 of which were congenital. Three cases of WPW were recorded in 699 patients with ventricular septal defects. Out of 1 348 cases of atrial septal defect, 5 cases of pre-excitation were recorded, including 3 right posterior pre-excitations associated with an ostium primum defect. Pre-excitation was also observed in isolated cases of corrected transposition of the great arteries, supravalvular aortic stenosis, aortic incompetence and patent ductus arteriosus. Pre-excitation and acquired heart disease: Five cases of pre-excitation were recorded out of 305 cases of dilated cardiomyopathy (1.62 p. 100). Eleven cases of pre-excitation were recorded in a total of 3 471 cases of valvular heart disease (0.31 p. 100): 9 in rheumatic valve disease and 2 in mitral valve prolapse. Nine cases of pre-excitation were observed in 2 850 cases of coronary artery disease. Intermittent Wolff-Parkinson-White syndrome: Ventricular pre-excitation masks the ECG changes of complete right bundle branch block in Ebstein's anomaly, complete left bundle branch block in aortic incompetence and dilated cardiomyopathy, and the in-complete right bundle branch block often seen in mitral valve prolapse. The characteristic appearances of WPW depend on the zone of pre-excitation. Right ventricular hypertrophy observed in ventricular septal defect with pulmonary stenosis and mitral stenosis may be masked by right lateral pre-excitation. Changes of inferior wall myocardial infarction may be masked by left anterior wall pre-excitation. On the other hand, the effects of WPW on left ventricular hypertrophy are variable, high amplitudes of the resultant forces seeming to depend on late and isolated activation of one of the left ventricular walls.     

Pattern of heart disease in Ethiopia as seen in a cardiology referral clinic

385 patients were seen in the cardiology clinic of Tikur Anbessa Hospital, Addis Ababa, Ethiopia over 20 months. Of 338 with defined pathology, 152 had rheumatic heart disease, 47 were hypertensive, 39 had cardiomyopathy, 36 had congenital heart disease and 24 arrhythmia. Average age of rheumatics was 25.5, 78% were less than or equal to 30, male:female = 58:94. The mitral valve was affected in 91%; 18% of rheumatics had pure mitral stenosis and 56% only mitral involvement. Average age of cardiomyopathy patients was 52, 90% had dilated cardiomyopathy. In congenital cases, mitral valve prolapse was most common (25%), followed by ventricular septal defect (19%), and patent ductus arteriosus (19%). Comparison is made with Ethiopian and other African data. Clearly, rheumatic fever is the main cause of cardiac pathology in Ethiopia, and deserves greatly increased attention.     

Detection of cardiovascular abnormalities in the nursery of a general hospital in the Amazon region: correlation with potential risk factors.

Congenital heart diseases have been studied much more extensively in children than in neonates. In this study, we report on the findings from 57 neonates seen from June of 1995 through June 1996 in the nursery of a large public hospital in Belém, Pará, Brazil. All were routinely examined by a paediatrician just after birth, and, when indicated, these babies were referred to the cardiology unit of our Hospital for assessment by a paediatric cardiologist. Most of the diagnoses were made by means of Doppler and cross-sectional echocardiography with color flow mapping. Several abnormalities of the cardiovascular system were diagnosed. The most frequent was patency of the arterial duct. But, since many ducts closed spontaneously, ventricular septal defect was the most frequent lesion seen even in the nursery. Four defects (patent arterial duct, ventricular septal defect, atrial septal defect and pulmonary stenosis) together accounted for two thirds of all cardiac abnormalities. Associated non-cardiac anomalies were more frequent in those with simple lesions within the heart. All the babies with complex heart disease, and the majority of those designated as having significant lesions, died before they could be discharged. Several risk factors were investigated. Among maternal drugs, misoprostol emerged as having a possible teratogenic effect.     

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.     

社区儿童保健体检272 例心脏杂音结果分析

目的:初步探讨社区卫生中心针对0~6 岁散居婴幼儿及儿童保健体检对疾病尤其心脏疾病筛查的重要性,以及心脏杂音体征在0~6岁散居婴幼儿和儿童心脏疾病诊断中的重要性。方法:对2009 年7月~2013 年6 月本社区卫生服务中心所属辖区进行0~6岁散居儿童保健大体检中听诊到心脏杂音的272名婴幼儿及儿童进行专案管理登记并尽快转至上级专科医院进行心脏彩超、心肌酶等检查以明确产生心脏杂音的原因及疾病。结果:转诊的272名查体有心脏杂音的0~6岁儿童上级心血管专科医院的诊断结果为病理性杂音124例,占45.6%,生理性杂音148例,占54.4%,其中病理性杂音包括先天性心脏病85例（占31.3%）,即室间隔缺损（VSD）62 例（占22.8%）、房间隔缺损（ASD）13 例（占4.8%）、房室间隔缺损（AVSD）2例（0.7%）,动脉导管未闭（PDA）10 例（3.7%）;继发性心脏病37例（占13.6%）,包括感染性心肌损害27 例（9.9%）、心肌炎2 例（0.7%）、感染性心内膜炎1例（0.4%）,贫血性心脏病7 例（25.7%）;生理性杂音148例,包括左室假腱索42 例（15.4%）、瓣膜反流及瓣膜脱垂42例（15.4%）,心脏无异常64 例（23.5%）。先天性心脏血管病<1岁年龄组所占比例显著高于1-岁组,更高于2-岁以上组（均P<0.01）。而继发性心脏病和生理性杂音<1岁组所占比例显著低于1 岁组和2 岁组（均P<0.01）。在<1岁组中先天性心脏血管病所占比例显著高于继发性心脏病和生理性杂音。结论:本社区儿童保健体检查体中对发现的异常体征如心脏杂音的婴幼儿及儿童进行及时专科转诊后发现先天性心脏病和继发性心脏病的比例较高,是引起心脏杂音的主要原因。社区卫生中心针对散居婴幼儿及儿童每年定期免费保健大体检可对散居儿童的生长发育和疾病筛查有重要意义和积极作用,认真做好基层医疗儿童保健工作可使散居儿童得到疾病的早发现、早治疗。     

Clinical features of hypertrophic cardiomyopathy in the young

BACKGROUND: We analysed the experience with hypertrophic cardiomyopathy in two paediatric centres to establish the differences from older patients. METHODS: Out of 45 young patients seen from 1974 to 1999, we included 38. Criterions for exclusion were secondary forms, or association with severe congenital cardiac disease which could alter the outcome. RESULTS: The patients presented at the age of 5.7 years, and were followed for 7.0 years. The 34 patients referred because of a murmur or cardiomegaly were older than the four with heart failure, presenting at 6.2 as opposed to 2.1 years of age, p = 0.08. Of the patients, 29 (76%) had primary cardiomyopathy, while 9 (24%) had secondary forms associated with Noonan's and LEOPARD syndromes. Familial tendency was ascertained in 7 patients (18%). The septal thickness in mm/m2 at presentation was greater in patients under 2 years than in older children (29 vs 18, p = 0.02). Obstructive hypertrophic cardiomyopathy was found in 17 patients (45%), with six of these having mild associated congenital cardiac defects. Nine had symptomatic arrhythmias. Overall, treatment was medical in 31, with DDD pacing used in 5, and surgery, radiofrequency ablation, and transplantation in one patient each. Total mortality was 24%, at a rate of 4.3% per year. Four patients died in heart failure and 5 had sudden death. Those in failure were significantly younger (p = 0.01). CONCLUSIONS: Hypertrophic cardiomyopathy in the young is characterized by referral for murmur or heart failure; frequent secondary forms; the obstructive variant being as common as the non-obstructive form; a mortality rate similar to that for adults attending tertiary centres; and less frequent familial forms than in older populations.     

The clinical spectrum of tricuspid regurgitation detected by pulsed Doppler echocardiography

The clinical diagnosis of tricuspid regurgitation (TR) is often difficult. Two-dimensional pulsed Doppler echocardiography offers a sensitive and specific method for detecting and semi-quantitating tricuspid regurgitation. The clinical, radiographic, radionuclide, echocardiographic, and when available, the right cardiac catheterization findings were evaluated in 36 patients with a diagnosis of tricuspid regurgitation by pulsed Doppler. Ten healthy subjects served as controls. The underlying cardiac cause was rheumatic heart disease in 7 (20%), ischemic heart disease in 12 (33%), dilated cardiomyopathy in 5 (14%), hypertensive heart disease in 2 (5%), aortic valve stenosis and/or regurgitation in 3 (8%), mitral valve prolapse with mitral regurgitation in 1 (3%), and congenital heart disease in 6 (17%). Seven patients (19%) had a temporary or permanent transvenous right ventricular pacing wire. A systolic murmur was heard in 29 patients (81%) with 16 (46%) having an elevated jugular venous pressure. Tricuspid regurgitation was clinically suspected in only 2 patients (6%). Isolated tricuspid regurgitation was uncommon, seen in 6 patients (17%), and usually secondary to congenital heart disease, ischemic heart disease, with the use of a transvenous pacing wire and following mitral valve replacement. Right cardiac catheterization was performed in 10 patients, of which 7 demonstrated elevated right atrial and pulmonary artery pressure. Pulsed Doppler echocardiography offers a practical and accurate method of detecting and evaluating the severity of tricuspid regurgitation. Tricuspid regurgitation is generally a functional disorder, and frequently occurs in association with left sided valvular heart disease, cardiomyopathy or congenital heart disease.