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.

A retrospective survey comparing suture techniques regarding the risk of permanent epicardial pacemaker implantation after ventricular septal defect closure

Abstract Objective: The aim of this study is to compare the continuous and combined suturing techniques in regards to the needing epicardial pacing at the time of weaning from cardiopulmonary bypass (EP-CPB) and to evaluate permanent epicardial pacemaker (PEP) implantation in patients who had undergone surgical ventricular septal defect (VSD) closure. Methods: This single-centre retrospective survey includes 365 patients who had consecutively undergone VSD closure between January 2006 and October 2015. Results: The median age and weight of the patients were 15 months (range 27 days - 56.9 years) and 10 kg (range 3.5 - 100 kg), respectively. Continuous and combined suturing techniques were utilised in 302 (82.7%) and 63 (17.3%) patients, respectively. While 25 (6.8%) patients required EP-CPB, PEP was implanted in eight (2.2%) patients. Comparison of the continuous and combined suturing techniques regarding the need for EP-CPB (72% vs. 28%, P=0.231) and PEP implantation (87.5% vs. 12.5%, P=1.0) were not statistically significant. The rate of PEP implantation in patients with perimembraneous VSD without extension and perimembraneous VSD with inlet extension did not reveal significant difference between the suture techniques (P=1.0 and P=0.16, respectively). In both univariate and multivariate analyses, large VSD (P=0.001; OR 8.63; P=0.011) and perimembraneous VSD with inlet extension (P<0.001; OR 9.02; P=0.005) had a significant influence on PEP implantation. Conclusion: Both suturing techniques were comparable regarding the need for EP-CPB or PEP implantation. Caution should be exercised when closing a large perimembraneous VSD with inlet extension.

Cardiovascular abnormalities in patients with oral cleft: a clinical-electrocardiographic-echocardiographic study

OBJECTIVES: The present study aims to describe the clinical, electrocardiographic, and echocardiographic cardiological findings in a group of patients with oral clefts. METHODS: This is a prospective cross-sectional study on 70 children (age range from 13 days to 19 years) with oral clefts who attended the multidisciplinary program of a university hospital from March 2013 to September 2014. The patients were evaluated by a pediatric cardiologist and underwent detailed anamnesis, physical examination, electrocardiogram, and echocardiogram. RESULTS: Sixty percent of the patients were male; 55.7% presented with cleft lip and palate, and 40.0% presented with health complaints. Comorbidities were found in 44.3%. Relevant pregnancy, neonatal, family and personal antecedents were present in 55.7%, 27.1%, 67.2%, and 24.3% of the patients, respectively. Regarding the antecedents, 15.2% of the patients presented with a cardiac murmur, 49.0% with a familial risk of developing plurimetabolic syndrome, and 6% with family antecedents of rheumatic fever. Electrocardiographic evaluation showed one case of atrioventricular block. Echocardiograms were abnormal in 35.7% of the exams, including 5 cases of mitral valve prolapse — one of which was diagnosed with rheumatic heart disease. CONCLUSION: The finding of a family risk of developing plurimetabolic syndrome and a diagnosis of rheumatic heart disease indicates that patients with oral clefts may be more prone to developing acquired heart disease. Thus, our findings highlight the importance of anamnesis and methodological triangulation (clinical-electrocardiographic-echocardiographic) in the investigation of patients with oral clefts and emphasize that cardiological follow-up to evaluate acquired and/or rhythm heart diseases is necessary. This strategy permits comorbidity prevention and individualized planned treatment.

Risk factors for delayed extubation after ventricular septal defect closure: a prospective observational study

Objective: The objective of our study was to determine the feasibility of early extubation and to identify the risk factors for delayed extubation in pediatric patients operated for ventricular septal defect closure. Methods: A prospective, observational study was carried out at our Institute. This study involved consecutive 135 patients undergoing ventricular septal defect closure. Patients were extubated if feasible within six hours after surgery. Based on duration of extubation, patients were divided two groups: Group 1= extubation time ≤ 6 hours, Group 2= extubation time >6 hours. Results: A total of 99 patients were in Group 1 and 36 patients in Group 2. Duration of ventilation was 4.4±0.9 hours in Group 1 and 25.9±24.9 hours in Group 2 (P<0.001). Univariate analysis showed that young age, low weight, low partial pressure of oxygen, trisomy 21, multiple ventricular septal defect, high vasoactive inotropic score, transient heart block and low cardiac output syndrome were associated with delayed extubation. However, regression analysis revealed that only trisomy 21 (OR: 0.248; 95%CI: 0.176-0.701; P=0.001), low cardiac output syndrome (OR: 0.291; 95%CI: 0.267-0.979; P=0.001), multiple ventricular septal defect (OR: 0.243; 95%CI: 0.147-0.606; P=0.002) and vasoactive inotropic score (OR: 0.174 95%CI: 0.002-0.062; P=0.039) are strongest predictors for delayed extubation. Conclusion: Trisomy 21, low cardiac output syndrome, multiple ventricular septal defect and high vasoactive inotropic score are significant risk factors for delay in extubation. Age, weight, pulmonary artery hypertension, size of ventricular septal defect, aortic cross-clamp and cardiopulmonary bypass time did not affect early extubation.

Henoch-Schonlein purpura secondary to infective endocarditis in a patient with pulmonary valve stenosis and a ventricular septal defect

No abstract available.

Permanent complete heart block following surgical closure of isolated ventricular septal defect

A serious complication after surgical closure of ventricular septal defect [VSD] is complete heart block [CHB]. It continues to be a leading cause of long-term postoperative cardiac morbidity despite all surgical technical improvements, especially with performance of more surgical procedures in increasingly younger patients. This study was undertaken to determine the incidence of early postoperative CHB requiring pacemaker implantation following surgical repair of isolated ventricular septal defect, and try to identify possible procedural risk factors. Prospective study design. Multicenter study: Ain-Shams University Hospital, Aboul Reesh Student Hospital and The National Heart Institute. We reviewed four hundred patients who had a surgical repair of isolated VSD from 2009 to 2011. 14 out of 400 patients [3.5%] developed permanent post-operative CHB. All; but one; underwent closure of large perimembranous VSD. CHB patients had a significant lower body weight [8.36 vs. 12.68 kg, p < 0.01], longer ACC time [42.6 vs. 36.4 min, p < 0.01], longer CPB time [75.4 vs. 67.4 min, p < 0.01] and longer hospital stay [19 vs. 8.3 days, p < 0.01] Tricuspid valve detachment was performed in 28 patients [7%] and was associated with a higher incidence of CHB [7% vs. 3.75%, p = 0.6]. Large perimembranous VSD and lower body weight appear to be independent risk factors for permanent CHB following the surgical closure of isolated VSD

Safe management of cesarean section in a patient of Eisenmenger syndrome.

We report our experience of a 29-year-old female with a complete atrio-ventricular septal defect leading to a single ventricle physiology and Eisenmenger syndrome. The patient successfully underwent spinal anesthesia for cesarean section in the 31 st week of pregnancy. A multidisciplinary approach involving cardiologist, cardiac surgeon, obstetrician, and anesthesiologist was utilized to achieve a safe pregnancy and cesarean for the delivery of the baby. A close clinical assessment is required, especially during the third trimester when the risk of acute right ventricular dysfunction increases. The use of extracorporeal membrane oxygenation (ECMO) (as a bridge to recovery or bridge to salvage) was planned to support oxygenation and circulation in case of acute biventricular dysfunction. The delivery/cesarean section was performed in a cardiac surgery operating room, and to reduce the time-frame for ECMO institution the femoral vessels were exposed surgically before the cesarean section.

Complicaciones del tratamiento quirúrgico de la comunicación interventricular en pacientes menores de 17 años en el INSN / Surgical and medical complications in ventricular septal defects surgical treatment in pediatrics patients in INSN

OBJETIVO: Conocer las complicaciones médico quirúrgicas, post tratamiento quirúrgico de la comunicación interventricular (CIV) de pacientes menores de 17 años, tratados en el INSN. MATERIAL Y MÉTODOS: Estudio descriptivo, retrospectivo. Se incluyeron todos los pacientes menores de 17 años atendidos por CIV en el INSN, de Enero del 2007 a Julio del 2011. RESULTADOS: Se revisaron 61 historias clínicas de pacientes con diagnóstico de CIV. 22 eran de sexo femenino (36.1%), y 39 masculino (63.9%). El tipo de CIV más frecuente fue el perimembranoso (78.7%) seguidos por los CIV de entrada de salida y muscular (7.1%). Según la técnica operatoria: 75.4% fueron transatriales y 11.5% transpulmonares. Las complicaciones médicas post quirúrgicas más frecuentes, fueron el bloqueo aurículo ventricular e insuficiencia cardiaca congestiva ambas con el 8.2% de frecuencia. La complicación quirúrgica más frecuente fue el CIV residual (29.5%), seguida de dehiscencia esternal (9.8%) e infección de herida operatoria (8.2%). 47.5% no presentó ninguna complicación quirúrgica. CONCLUSIONES: El tratamiento quirúrgico de la comunicación interventricular es una alternativa segura para la corrección de esta patología.

OBJETIVE: The purpose of this study was to know the surgical and medical complications in ventricular septal defects (VSD) surgical treatment in pediatrics patients in INSN. MATERIALS AND METHODS: A descriptive and retrospective study in pediatrics patients that underwent VSD surgical closure in the cardiac service of ôINSNõ in Lima, Perú, between January 2007 to July 2011. RESULTS: We reviewed 61 medical records, there were 22 (36.1%) females and 39 (63.9%) males. The perimembranous VSDs was the more frequent (78.7%) followed the enter and way muscle VSDs (7.1%). The closure of the ventricular septal defects with the transatrial approach was the predominant surgical treatment (29.5%). The residual shunts were the complication in the surgical management of VSDs with more incidence. The auricular-ventricular block and congestive heart failure were the medical complications with more incidence (both with 8.2%). 47.5% of patients had no surgical complications. CONCLUSIONS: The ventricular septal defects surgical closure is the safe alternative for correction this anomaly in the INSN.

Comunicación interventricular en el feto / Intraventricular communication in fetus

Se reporta el caso de una gestante que en ultrasonido realizado en el segundo trimestre de la gestación se sospecha un defecto de septación ventricular, se confirmó la presencia de una comunicación interventricular tipo perimembranosa con extensión muscular y mal alineamiento del tabique interventricular, que trajo como consecuencia disminución del tamaño del tracto de salida del ventrículo izquierdo, sin causar obstrucción. En el estudio posnatal se evidenció la patología señalada así como la presencia de una comunicación interauricular y persistencia del conducto arterioso. Las ilustraciones empleadas se corresponden con imágenes grabadas en el Cardiocentro Pediátrico William Soler y en su servicio de anatomía patológica. Se trata del primer caso reportado en nuestro país

The case of a pregnant with suspicion of a ventricular septate defect according to ultrasound carried out during the second trimester of pregnancy is reported confirming the presence of a peri-membranous intraventricular communication with muscular lengthening and a malalignment of interventricular septum leading to a decrease in the size of outlet tract from the left ventricle without obstruction. In the postnatal study it was evidenced the marked pathology, as well as the presence of a interauricular communication and persistence of arterial conduct. Illustrations used are in correspondence with the images taped in the William Soler Children Heart Center and in its pathological anatomy service

Oclusão percutânea de comunicação interventricular por via arterial: técnica alternativa / Retrograde transcatheter occlusion of ventricular septal defects: an alternative technique

Introdução: A oclusão percutânea das comunicações interventriculares (CIVs) surgiu como uma alternativa ao tratamento cirúrgico ainda na década de 1980. Durante o implante da prótese por via anterógrada, a formação de uma alça arteriovenosa com a guia é tecnicamente difícil e demanda tempo. O objetivo deste estudo foi apresentar a experiência dos autores com a via retrógada e mostrar sua exequibilidade nos diversos tipos de CIV. Métodos: Foram incluídos pacientes portadores de defeitos do septo interventricular, que não apresentassem outros defeitos associados. Os casos foram selecionados por meio de ecocardiografia transtorácica. Resultados: No total, 8 pacientes foram submetidos ao procedimento, 4 portadores de CIV muscular e 4 portadores de CIV perimembranosa. Metade era do sexo masculino, as idades variaram de 7 anos a 32 anos (18,6 ± 7,8 anos) e os pesos, de 30 kg a 97 kg (66,12 ± 24,7 kg). Os diâmetros dos defeitos variaram de 2 mm a 8,6 mm (5 ± 2,4 mm). O implante foi possível em todos os casos, e a prótese mais utilizada foi a Lifetech CeraTM tipo I. As próteses AmplatzerTM Muscular VSD Occluder e Lifetech CeraTM tipo II também foram empregadas. Duas próteses embolizaram imediatamente após o implante e foram retiradas. Oclusão imediata ocorreu em 75%. Uma paciente apresentou bloqueio atrioventricular total na semana seguinte ao procedimento. Foi tratada com esteroides, obtendo a reversão para ritmo sinusal. Conclusões: A utilização da via retrógrada mostrou-se eficaz na oclusão de todos os tipos morfológicos de CIV com próteses de duplo disco. Os autores sugerem essa técnica como alternativa para a oclusão percutânea das CIVs de qualquer morfologia.

BACKGROUND: Transcatheter occlusion of ventricular septal defects (VSDs) was developed as an alternative to surgical treatment in the 80's. During anterograde device implantation, the formation of an arteriovenous loop with a guide-wire is technically difficult and time consuming. The aim of this study is to present the authors' experience with the retrograde approach and demonstrate its feasibility in different types of VSD. METHODS: Patients with VSD with no other associated defects were included in the study. Cases were selected by transthoracic echocardiograms. RESULTS: A total of eight patients were submitted to the procedure, 4 with muscular VSD and 4 with perimembranous VSD. Half of them were male, with ages ranging from 7 to 32 years (18.6 ± 7.8 years) and weight ranging from 30 to 97 kg (66.12 ± 24.7 kg). The defect diameter ranged from 2 to 8.6 mm (5 ± 2.4 mm). Implantation was possible in all patients and the most commonly used device was Lifetech CeraTM Type I. AmplatzerTM Muscular VSD Occluder, and Lifetech CeraTM Type II devices were also used. Two devices embolized immediately after implantation and were snared out. There was immediate occlusion in 75% of the cases. One patient had a complete atrioventricular block in the week following the procedure. The patient was treated with steroids, and was reverted to normal sinus rhythm. CONCLUSIONS: Retrograde transcatheter VSD occlusion with double disk devices proved to be effective in different types of VSD. The authors suggest this technique as an alternative to percutaneous occlusion of all types of VSD.

Comunicação interventricular pequena: conduta clínica expectante em longo prazo / Comunicación interventricular pequeña: conducta clínica expectante a largo plazo / Small ventricular septal defect: long-term expectant clinical management

FUNDAMENTO: A comunicação interventricular (CIV) pequena apresenta geralmente boa evolução clínica, mesmo em longo prazo. OBJETIVO: Verificar evolução clínica de pacientes com CIV pequena, a fim de determinar continuidade ou não da conduta expectante, em vista do baixo risco operatório, o que ocasiona maior liberalidade da indicação cirúrgica. MÉTODOS: No período de outubro de 1976 a dezembro de 2007, foram examinados 187 casos com CIV pequena (diâmetro menor que 3 mm pelo ecocardiograma) e seguidos 155 deles em longo prazo. Estudaram-se a época de exteriorização do sopro e aspectos evolutivos como fechamento espontâneo do defeito (grupo I) - 64 casos, continuidade do tamanho inicial (grupo II) - 74 casos e diminuição do mesmo (grupo III) -17 casos, além de intercorrências clínicas. RESULTADOS: A exteriorização do sopro ocorreu na maioria no primeiro mês de vida, correspondendo a 48 (75 por cento), 54 (72,9 por cento) e 12 (70,5 por cento) pacientes, nos três grupos respectivos, e acima do primeiro ano em 11 (5,8 por cento) pacientes. Fechamento espontâneo ocorreu no primeiro ano em 48 casos (75 por cento), média de 7,6 m e de 1 a 5,5 anos em 15 pacientes (23,4 por cento), em acompanhamento máximo de 18 anos. Verificou-se continuidade do defeito até 40 anos. A diminuição do defeito ocorreu em média de 15 meses, seguidos até 9 anos. Probabilidade de fechamento da CIV, pela curva actuarial, foi de 34,38 por cento em 1 ano e de 49,89 por cento em 5 anos. Não houve intercorrências clínicas. CONCLUSÃO: Evolução favorável da CIV pequena em longo prazo dispensa intervenção operatória, com preocupação da profilaxia antibiótica rigorosa.

BACKGROUND: The small ventricular septal defect (VSD) usually presents good clinical evolution, even at long-term follow-up. OBJECTIVE: To verify the clinical evolution of patients with small VSD in order to determine the continuation or not of the expectant conduct, considering the low operative risk, which results in a more liberal indication for surgery. METHODS: From October 1976 to December 2007, 187 cases of small VSD (diameter < 3 mm at the echocardiogram) were evaluated and 155 of them were assessed at long-term follow-up. Time of the clinical manifestation of the murmur and evolution aspects such as the spontaneous closure of the defect (group I) - 64 cases, persistence of the initial size (group II - 74 cases and decrease in the size of the defect (group III) - 17 cases, in addition to clinical complications, were studied. RESULTS: The clinical manifestation of the murmur occurred, in the majority of cases, during the first month of life, corresponding to 48 (75 percent), 54 (72.9 percent) and 12 (70.5 percent) patients, in the three groups, respectively and after the first year of life in 11 (5.8 percent) patients. Spontaneous closure occurred in the first year of life in 48 cases (75 percent), mean of 7.6 months and from 1 to 5.5 years in 15 patients (23.4 percent), with a maximum follow-up of 18 years. The persistence of the defect until 40 years of age was observed. The decrease in the size of the defect occurred on a mean of 15 months, followed for up to 9 years. The probability of VSD closure by the actuarial curve was 34.38 percent in 1 year and 49.89 percent in 5 years. There were no clinical complications. CONLCUSION: A favorable evolution of the small VSD at long-term follow-up does not require surgical intervention, with concerns regarding the strict antibiotic prophylaxis.

FUNDAMENTO: La comunicación interventricular (CIV) pequeña presenta generalmente buena evolución clínica, aun a largo plazo. OBJETIVO: Verificar la evolución clínica de pacientes con CIV pequeña, a fin de determinar continuidad o no de la conducta expectante, en vista del bajo riesgo operatorio, resultando una mayor liberalidad de la indicación quirúrgica. MÉTODOS: En el período de octubre de 1976 a diciembre de 2007, se examinaron 187 casos con CIV pequeña (diámetro menor que 3 mm por el ecocardiograma) y se siguieron a 155 de ellos a largo plazo. Se estudiaron la época de exteriorización del soplo y los aspectos evolutivos, como cierre espontáneo del defecto (grupo I): 64 casos; continuidad del tamaño inicial (grupo II): 74 casos; y disminución del mismo (grupo III):17 casos; además de intercurrencias clínicas. RESULTADOS: En la mayoría de los casos, la exteriorización del soplo tuvo lugar en el primer mes de vida, correspondiendo a 48 (75 por ciento), 54 (72,9 por ciento) y 12 (70,5 por ciento) pacientes, en los tres grupos respectivos, y después del primer año en 11 (5,8 por ciento) pacientes. Cierre espontáneo ocurrió en el primer año en 48 casos (75 por ciento), promedio de 7,6 m y de 1 a 5,5 años en 15 pacientes (23,4 por ciento), en seguimiento máximo de 18 años. Se verificó continuidad del defecto hasta 40 años. La disminución del defecto ocurrió en promedio de 15 meses, seguidos hasta 9 años. Probabilidad de cierre de la CIV, por la curva actuarial, fue de un 34,38 por ciento en 1 año y de un 49,89 por ciento en 5 años. No hubo intercurrencias clínicas. CONCLUSIÓN: Evolución favorable de la CIV pequeña a largo plazo dispensa intervención operatoria, con preocupación de la profilaxis antibiótica rigurosa.

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.

Comunicação interventricular: pequenos defeitos, grandes complicações / Ventricular septal comunications: small defects, big complications

Não existe um consenso definitivo sobre o tratamento cirúrgico de pequenos defeitos do septo ventricular com apresentação clínica assintomática. Embora estes defeitos congênitos apresentem evolução benigna, com fechamento espontâneo, em alguns casos podem ser observadas grandes complicações decorrentes do próprio mecanismo de fechamento ou do aparecimento de novas lesões na evolução da doença.

Interrupção do arco aórtico como causa de insuficiência cardíaca em adulto jovem / Interrupted aortic arch with cardiac heart failure in young adult

A interrupção do arco aórtico é uma cardiopatia rara, com alta morbidade e mortalidade quando o manejo é retardado. Este artigo apresenta um caso com evolução atípica, em paciente de 19 anos. A história e o exame clínico eram compatíveis com insuficiência cardíaca recente, associados a episódios de síncope e diminuição de pulsos em membros inferiores. O eletrocardiograma mostrava sobrecarga biventricular e biatrial. O ecocardiograma mostrou disfunção severa dos ventrículos e alteração de fluxo em aorta descendente. Na angiotomografia, visualizou-se oclusão da aorta torácica após a artéria subclávia esquerda. O diagnóstico instituído foi de interrupção de aorta, sendo indicado tratamento cirúrgico.

The interrupted aortic arch (IAA) is a rare cardiopathy, with high morbimortality when treatment is delayed. This study is a case of IAA with atypical clinical behavior in a 19-year-old patient. The history and the clinical assessment were compatible with recent heart failure, associated to syncope episodes and reduction of pulses in the lower limbs. The electrocardiogram showed biventricular and biatrial overload. The echocardiogram showed severe ventricular dysfunction and altered blood flow in the descending aorta. The angiotomography disclosed occlusion of the thoracic aorta after the left subclavian artery. The attained diagnosis was interrupted aortic arch and surgical treatment was indicated.