Syndrome of ventricular septal defect and aortic regurgitation - A 22-year review of its management

Abstract Introduction: The presence of aortic regurgitation (AR) in the setting of ventricular septal defect (VSD) has always been a management challenge. Methods: This is a retrospective study looking at patients who underwent VSD closure with or without aortic valve intervention between January 1st, 1992 and December 31st, 2014 at the Institute Jantung Negara. This study looked at all cases of VSD and AR, where AR was classified as mild, moderate, and severe, the intervention done in each of this grade, and the durability of that intervention. The interventions were classified as no intervention (NI), aortic valve repair (AVr), and aortic valve replacement (AVR). Results: A total of 261 patients were recruited into this study. Based on the various grades of AR, 105 patients had intervention to their aortic valve during VSD closure. The rest 156 had NI. All patients were followed up for a mean time of 13.9±3.5 years. Overall freedom from reoperation at 15 years was 82.6% for AVr. Various factors were investigated to decide on intervening on the aortic valve during VSD closure. Among those that were statistically significant were the grade of AR, size of VSD, age at intervention, and number of cusp prolapse. Conclusion: We can conclude from our study that all moderate and severe AR with small VSD in older patients with more than one cusp prolapse will need intervention to their aortic valve during the closure of VSD.

morphological classification of ventrical septal defects

The ventricular septal defects are the most common cnongenital heart disease, Ventricular septum composed of two parts:the memberanous septum is relatively small very thin stracture and themuscular septum, Which is divided into three regions; inlet; trabecular and outlet portion. To find out morphological classification of ventricular septal defects in Iraqi population. Two hundred isolated ventricular septal defect in 196 patients, were identified by two dimension echocardiography and Doppler color flow mapping. Age, Sex of patients and family history were studied. Cross section scan was obtained using a SSH-140A Toshiba machine. Ventricular septal defects were perimemberanous in 70%, inlet in 18%, Trabecular in9%, and outlet in 3%. Lage defects were identified in 21%, and eisenmengers cpmplex in 5%. Delay in referreal of patient with ventricular septal defects to our hospital usually resulted in delay institusion of appropriate management and increase risk of future surger

Pattern of ventricular Septal defects and associated complications

To determine the frequency of various types of Ventricular Septal Defect [VSD] in local population and their complications. Case series. The Pediatric Cardiac Unit of the Children Hospital and Institute of Child Health, Lahore, from January 2006 to December 2007. All patients aged between one day to 18 years presenting to the unit with isolated VSD during the study period were evaluated using 2-D, continuous wave Doppler and colour Doppler techniques on transthoracic echocardiography. Where necessary, transesophageal echocardiography and/or angiography data was used to further clarify the anatomy. Of the 854 patients with isolated VSD, 710 were of perimembranous type [83.1%], 100 were muscular type [11.7%], 25 were doubly committed subarterial [DCSA] type [2.9%] and 19 patients were having inlet VSD [2.2%]. The mean age was 2.1 +/- 3 years. Females were 332 [38.8%] and males were 522 [61.1%]. Aortic Valve Prolapse [AVP] was seen in 77 patients [10.8%]. The mean age for this subgroup was 5.8 +/- 4 years with 54 [70.1%] males and 23 [29.9%] females. Of those, 36 [47.3%] had various degree of aortic regurgitation. Right ventricular outflow tract obstruction of variable severity was found in 4 patients. Two of these patients had echocardiographic evidence of subacute bacterial endocarditis. Perimembranous [PM] VSD is the commonest type of ventricular septal defect presenting to a tertiary care hospital. The incidence of AVP and AR is high [10.8%] and was found almost entirely in perimembranous outlet VSDs rather than DCSA. This observation has special implication in Pakistan where pool of unoperated VSDs is large

Natural aortic valve complications of ventricular septal defect: a prospective cohort study.

OBJECTIVE: To study the incidence and onset of aortic valve prolapse (AVP) and aortic regurgitation (AR) in the ventricular septal defect (VSD). STUDY DESIGN: A prospective cohort study POPULATION: The less than one-year-old children with diagnosis of isolated VSD were studied from October 2000 to September 2006 at Queen Sirikit National Institute of Child Health. Clinical follow-up and echocardiographic studies were scheduled every 2-3 months in the first year of age and then every 6 months to evaluate the size, location, flow across VSD, aortic valve morphology and aortic regurgitation. RESULTS: Three hundred and twenty-one cases of VSD were followed up. One was excluded due to associated hypoplastic RV An overall of 2,644 echocardiograms were performed. The percentage of perimembranous, subpulmonic, muscular inlet and multiple types were 70.3%, 19.4%, 5.6%, 3.1% and 1.6%, respectively. Size of the VSD was diagnosed to be small, moderate, and large VSD in 62.5%, 15.9% and 21.6% respectively. At the end of the study, the incidence of AVP in subpulmonic VSD was 87.1% compared to 16.4% in perimembranous VSD, with a relative risk of 5.30 and the incidence of AR in subpulmonic VSD was 37.1% compared to 5.3% in perimembranous VSD, with a relative risk of 6.95. From the survival analysis, the patient with subpulmonic VSD developed AVP at 46%, 77%, 90% and 94% compare to 8%, 13%, 20% and 23% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). The patient with subpulmonic VSD developed AR at 8%, 17%, 35% and 38% compare to 2%, 4%, 5% and 7% of perimembranous VSD at 12, 24, 36 and 48 months of age respectively (p < 0.001). At the end of the study, ninety-six cases (30%) underwent cardiac operation with the indication of heart failure or the occurrence of AR. Sixty one cases (19.1%), including two cases of subpulmonic type had spontaneous closure of VSD. Seven cases (2.2%) had lost to follow up and five cases (1.6%) died during the follow up period. CONCLUSION: The incidence of AVP and AR are high in subpulmonic VSD being much higher than perimembranous VSD with a relative risk of 5.30 and 6.95 respectively. These complications are significantly from infancy period and are an indication for early cardiac surgery.

Experience on transplantation of auto-pulmonary graft treating with congenital aortic disease / 中华外科杂志

<p><b>OBJECTIVE</b>To summarize the experience on auto-pulmonary transplantation (Ross procedure) treating with congenital aortic disease.</p><p><b>METHODS</b>From October 1994 to November 2003, 20 cases of Ross procedure were performed to treat with congenital aortic disease, Male: 15 cases; Female: 5 cases; age: 25 years;</p><p><b>DIAGNOSIS</b>congenital heart disease (CHD), aortic abnormalities: 12 cases; aortic valve prolapse: 5 cases; aortic valve hypogenesis: 3 cases; combined with subacute bacterial endocarditis (SBE): 4 cases, and ventricle septal defect (VSD): 2 cases; UCG showed aortic stenosis(AS) and/or aortic insufficience (AI) (moderate to severe), Left ventricle diastole diameter (LVDD): (60.51 +/- 11.87) mm, the grade pressure across aortic valve: (27.04 +/- 6.80) mmHg, heart function (NYHA): Class II: 13 cases; Class III: 3 cases; all cases were performed under CPB and moderate hypothermia, the operation procedure was following: (1) taking off auto-pulmonary artery valve; (2) removing dysfunctional aortic valve and auto-transplantation of pulmonary valve on aortic root; (3) putting a pulmonary homograft to rebuild right ventricular outflow tract.</p><p><b>RESULTS</b>The mortality was 0 during stay at hospital, aortic valve function were all normal, LVDD decreased significantly (t = 3.4007, P = 0.0008), the grade pressure across aortic valve was in normal limitation, (6.8 +/- 0.19) mmHg. Follow-up showed heart function was in Class I (NYHA), aortic and pulmonary valve function was very well.</p><p><b>CONCLUSION</b>Ross procedure is a kind of effective alterative operation for treating with congenital aortic valve disease, with good short and middle term results.</p>

Aoric Valve Lesion in Type I Ventricular Septal Defect / 대한흉부외과학회지

BACKGROUND: In this study, we investigated the risk factors for the development or progression of aortic regurgitation(AR) in patients with type I ventricular septal defect (VSD) to determine the optimal surgical timing and strategy. MATERIAL AND METHOD: Three-hundred and ten patients with type I VSD with or without AR were included. The mean of age was 73.7+/-114.7 (1~37) months. One hundred and eighty six patients (60%) had no AR, 83 (27%) had mild AR, 25 (8%) had moderate AR and 16 (5%) had severe AR. Aortic valve was repaired in 5 patients and replaced in 11 patients with closure of VSD in the first operation. Four patients required redo aortic valve repair and 11 patients required redo aortic valve replacement. Age at operation, association with aortic valve prolapse, Qp/Qs, systolic pulmonary arterial pressure, VSD size and systolic pulmonary artery to aortic pressure ratio(s[PAP/AP]) were included as risk factors analysis for the development of AR. The long-term result of aortic valve repair and aortic valve replacement were compared. RESULT: Older age at operation, association with aortic valve prolapse, high Qp/Qs, and s[PAP/AP] were identified as risk factors for the development of AR (p<0.05, Table 2). The older the patient at the time of operation, the higher the severity of preoperative AR and the incidence of postoperative AR (p<0.05, Table 1, Fig. 1). For the older patients at operation, aortic valve repair had higher occurrence of AR compared to those who had aortic valve replacement (p<0.05, Fig. 2). CONCLUSION: From the result of this study, we can concluded that early primary repair is recommended to decrease the progression of AR. Aortic valve repair is not always a satisfactory option to correct the aortic valve pathology, which may suggest that aortic valve replacement should be considered when indicated.

Hemodynamic Status of Subpulmonic Ventricular Septal Defect in Relation to Morphologic Changes of Aortic Valve

PURPOSE: Subpulmonic ventricular septal defect is clinically important because the risks of aortic regurgitation and endocarditis are substantially increased. This study was conducted to evaluate the effect of hemodynamic changes induced by structural anomalies of aortic valve on the surgical outcome. METHODS: This clinical study was done by reviewing the medical records of 124 patients who were diagnosed and surgically managed at the Cardiovascular Center, Yonsei University, College of Medicine, from May 1991 to December 1994 were performed due to subpulmonic ventricular septal defect. RESULTS: The ages were between 4 months to 21 years 9 months(mean 4 years 10 months) and the sex ratio was 1.8:1 (male:female; 80:44). Patients were stratified according to the status of the aortic valves. Group 1 was comprised of those without aortic valve prolapse and aortic regurgitation, group 2 was comprised of those with only aortic valve prolapse, and group 3 had only aortic regurgitation, and group 4 had both conditions. The mean age, pulmonary arterial pressure, aortic diameter were statistically higher in group 3, those which only aortic regurgitation(p<0.05). On surgical exploration, aortic valve prolapse was found in 68(55%), and aortic regurgitation was found in 21(17%). Aortic valve prolapse was correlated with the increase in age(p<0.05), and the development of aortic valve prolapse and aortic regurgitation were statistically correlated with the decrease in pulmonary arterial pressure(p<0.05). When 108 patients were classified into 3 groups according to the degree of aortic valve prolapse and hemodynamic status around the inlet and outlet of right ventricle, higher the degree of aortic valve prolapse, the intraventricular flow was directed to inlet(p<0.05). CONCLUSIONS: Untreated subpulmonic VSD will result in aortic regurgitation and valve prolapse, thus the surgical correction is beneficial, especially performed at early age. Therefore effort should be made to carefully evaluate all patients with VSD, and if subpulmonic VSD is discovered, immediate surgical correction is should be performed.

Transesophageal Echocardiography in the Detection of Intracardiac Source of Cerebral Emboli

BACKGROUND: Intracardiac pathology resulting in embloic phenomena is a well-recognized cause of cerebral ischemia and infarction. Recently, the use of transesophageal echocardiography(TEE) has gained wide acceptance because of its superior resolution of basal structures such as the left atrium, left atrial appendage, mitral valvular apparatus, atrial septum, and aorta. The purposes of this study are to evaluate the effectiveness of TEE for detection of intracardiac source of cerebral emboli. METHOD: From 1991 to 1995, 215 patients were included in this study. All patients underwent both transthoracic and transesophageal imaging with saline contrast administration and Doppler color flow imaging. The study group consisted of 132 men and 83 women with a mean age of 51 years(range 15-74). We also reviewed TEE result of all patients according to two groups, which were divided by the presence of clinical cardiac abnormalities. RESULT: 1) TEE identified a potential cardiac source of embolism in 43.7%(94 to 215) of the overall study group compared with only 3.7%(8 to 215) by TTE. 2) Success rate of TEE was 93.5%(201 to 215). 3) Abnormalities noted by TEE included 23 patients with LA spontaneous echo contrast, 20 patients with patient foramen ovale, 16 patients with aortic atheroma, 16 patients with LA thrombi, 8 patients with atrial septal aneurysm, 5 patients with aortic valve prolapse, 4 patients with mitral valve prolapse, and 2 patients with LV thrombi. 4) In the 62 patients with cardiac disease, TEE identified 16 patients with LA spontaneous echo contrast, 12 patients with LA thrombi, 2 patients with patent foramen ovale, 1 patient with aortic atheroma, 1 patient with atrial septal aneurysm and 1 patients with aortic valve prolapse. In the 152 patients with no cardiac disease, TEE identified 18 patients with patent foramen ovale, 15 patients with aortic atheroma, 7 patients with artial septal aneurysm, 7 patients with spontaneous echo contrast, 4 patients with aortic valve prolapse, 1 patient with mitral valve prolapse and 1 patient LA thrombi. CONCLUSION: TEE was very useful method in investigating potential intracardiac source of cerebral emboli. Thus, the use TEE combined with TTE in patients with unexplained stroke should be recommended.

Clinical Study of Subarterial Ventricular Septal Defect

PURPOSE: The incidence of subarterial ventricular septal defect(SA VSD) ranges 25-30% among oriental patients with VSDs, which is greater than 5% reported in western. Natural history of the disease is characterized by progressive aortic valve prolapse(AVP), frequently subarterial VSD, we evaluated clinical characteristics emphasizing on the incidence of AVP and the degree of AI as aging. METHODS: Study subjects consisted of 140 patients, who were diagnosed as subarterial VSD and operated in Seoul paik Hospital during a 5 year period from Jan.1988 to Dec. 1992. The data were analyzed detrospectively as to clinical profiles, data of cardiac catheterization, frequencies of AVP, and AI in 5 each age group, operative methods, postoperative complications and mortality. RESULTS: The incidence of subarteial VSD was 34.6% of total operated VSD cases. Data of preoperative cardiac catheterization showed mean values of Qp/Qs and systolic pulmonary artery pressure, 1.43+/-0.47 and 33.8x16.4mmHg in each. Aortic valve prolapses and aortic insufficiencies were observed in 70.0% and 20.7% among patients, which showed increasing tendencies as ages increased. As operative methods, patch closures through main pulmonary artery were done mainly. In mild cases without AI or with grade I AI, simple VSD closures were performed but in more a advanced cases, 10 aortic valvuloplasties and additional 2 aortic valve replacements were performed. Total mortality rate was 2.1%. CONCLUSIONS: In the management of subarterial VSD, early elective closure regardless of shunt volume is important to prevent progressive aortic valve prolapse leading to aortic insufficiency.

Ventricular septal defect with aortic regurgitation: a hemodynamic and angiographic profile in Indian subjects.

The records of 362 patients of Ventricular Septal Defect (VSD) were analysed to find out the incidence of aortic regurgitation (AR) and their hemodynamic and angiographic features. Thirty-seven patients (10.2%) were found to have AR, whose mean age was 13.4 years (range: 2-45) and male to female ratio was 5:1. Of the 37 cases 31 (84%) had infracristal and 6 (16%) had supracristal VSD. In 31 patients with infracristal VSD the prolapsing cusp was Right Coronary Cusp (RCC) in 14 (48%), Noncoronary Cusp (NCC) in 12 (41%) and both RCC and NCC in 3 (11%). Of the 6 patients with supracristal VSD the prolapsing cusp was RCC in 5 (83%) and NCC in 1 (17%). In two patients the AR was due to bicuspid aortic valve. The pulmonary artery pressure was normal in 26 of 37 (70.2%) patients and the left to right shunt was 1.5:1 or less in 23 of 37 (62%) patients. Nineteen of the 37 patients (51.3%) had grade I or II AR and the remaining 18 (48.7%) had grade III or IV AR. There was no relationship between the severity of AR and the location of the VSD. In conclusion, in this series, the incidence of VSD+AR is relatively higher and that of supracristal VSD is lower. In majority of patients the left to right shunt is small and pulmonary artery pressure within normal limits. The prolapse of RCC is more common in supracristal VSD and there is no relation between the severity of AR and the location of the VSD.

Prevalência de prolapso valvar tricúspide e aórtico em portadores de prolapso valvar mitral / Prevalence of tricuspid and aortic valve prolapse in patients with mitral valve prolapse

Foram estudados, através da ecocardiografia bidimensional, 30 pacientes portadores de prolapso da valva mitral, sendo 15 do sexo feminino e 15 masculino, com idade média de 33,3 anos, com o objetivo de observar a prevalência de concomitante prolapso valvar tricúspide e aórtico. O prolapso da valva tricúspide foi obsrvado em 43,3% dos pacientes (13/30 anos, sendo que o folheto anterior e septal estava acometido em 92,3% e o posterior em 15,3% dos casos. A prevalência de prolapso da valva aórtica foi de 10% dos casos (3/30), estando ambos os folhetos avaliados - coronariano direito e näo coronariano - acometidos. Todos os pacientes com prolapso valvar aórtico apresentavam acometimento dos dois folhetos mitrais e de, no mínimo, dois folhetos tricuspídeos. Conclui-se que é comum o acometimento simultâneo das valvas tricúspide e aórtica em pacientes portadores de prolapso da valva mitral

Combined Mitral and Aortic Valve Prolapse

Identification of patients with combined valvular prolapse has important clinical imlications, because such patients appear to be early surgical candidates. Detection of combined valvular prolapse became more feasible with development of 2-dimentional echocardiography and the incidence of combained mitral and valve prolapse is reported to be variable from 3% to 24%. The authors found a case of combined mitral and aortic valve prolapse detected by 2-dimensional echocardiography. This 30-years-old male patient who admitted because of peptic ulcer bleeding revealed a prolapse of anterior mitral leaflet with regurgitation and also a prolapse of the right coronary cusp into the left ventricular outflow tract but without evidence of aortic regurgutation by Doppler echocardiogram. he discharged without surgical intervention and needs further observation.