Arrhythmia in Adult Congenital Heart Disease

This case report highlights the significance of a multidisciplinary approach in the management of patients with repaired membranous ventricular septal defect (VSD) who develop postoperative arrhythmias. We present the case of a young female who experienced symptomatic episodes of supraventricular tachycardia following VSD repair. Through electrophysiological study and radiofrequency ablation, multiple tachycardia substrates were identified and successfully ablated. This report underscores the importance of combining surgical repair, electrophysiological evaluation and intervention to achieve optimal outcomes in this specific patient population.

A Case of Intermediate Type Atrioventricular Septal Defect Associated with a Membranous Ventricular Septal Aneurysm in a 70-Year-Old Female Patient / 日本心臓血管外科学会雑誌

The case was a 70-year-old female. Atrioventricular septal defect was diagnosed in her childhood, however, surgical treatment had not been performed. The patient had suffered from heart failure at the age of 69, and she was referred to our hospital for treatment. Her diagnosis was intermediate type atrioventricular septal defect, moderate left atrioventricular valve regurgitation, membranous ventricular septal aneurysm and atrial flutter. An autologous pericardial patch was used to close the ostium primum type atrial septal defect associated with simultaneous covering of membranous ventricular septal aneurysmal wall. Concomitant left and right atrioventricular valvuloplasty and arrhythmia surgery were performed. Her postoperative course was uneventful and the patient was discharged from our department on the 16th postoperative day. To our knowledge, there are few reports of surgery for incomplete type atrioventricular septal defect in the elderly and no report for intermediate type atrioventricular septal defect in Japan. In incomplete type atrioventricular septal defect, symptoms such as supraventricular arrhythmia and heart failure develop according to aging. Reported surgical results in the elderly are quite good, and improvement of excise tolerance is expected. Precise evaluation and proper indication of surgical treatment is mandatory even in older patients.

A Case of Partial Anomalous Pulmonary Venous Return in an Adult Patient / 日本心臓血管外科学会雑誌

Most cases of partial anomalous pulmonary venous return are associated with atrial septal defect. We however report a surgical case of partial anomalous pulmonary venous return that was diagnosed in an adult without atrial septal defect. The patient was a 44-year-old man who presented with hemoptysis. Computed tomography revealed an absent left pulmonary artery. We performed bronchial artery embolization. Partial anomalous pulmonary venous return (Qp/Qs 3.33) in which the right pulmonary vein returned to the superior vena cava and moderate aortic regurgitation were diagnosed by computed tomography and transthoracic echocardiography ; this was an indication for surgery. Approximately 2 months after the emergency hospitalization due to hemoptysis, we performed a modified Warden procedure and aortic valve replacement. Postoperative computed tomography showed good reconstruction of the superior vena cava and right upper pulmonary vein. He was discharged on postoperative day 38.

Clinical, hemodynamic, echocardiographic, angiographic profiles and post-operative outcomes among DCRV patients from a tertiary care referral center in India

Background: This retrospective study of data from 2006 to 2018 at a tertiary care referral center in India aims to document the contemporary clinical and hemodynamic profile of patients who were diagnosed with double chamber right ventricle (DCRV) based on echocardiography and cardiac  catheterization. Patients were followed up and their outcomes were studied to document their short and long term outcomes.Methods: It is a retrospective observational study of patients diagnosed with DCRV in a tertiary care hospital in India. The diagnosis of DCRV was based on the following criteria: an echocardiographic diagnosis of DCRV with doppler evidence of a mid ventricular gradient; cardiac catheterization revealing a systolic pressure gradient between right ventricular inflow and outflow tracts; a right ventricular angiogram demonstrating an anomalous muscle bundle causing obstruction well below the infundibulum. All the patients were followed up for their long term outcomes.Results: All the patients underwent echocardiography and cardiac catheterization for confirmation of the diagnosis. Sixty percent of our patients presented during adulthood, which is very unusual presentation of this disease entity. Median age of our patient cohort was 23.5 years. Patients presenting during adulthood have atypical symptoms. Dyspnea was the most common presenting symptom in this study. Right ventricular hypertrophy (73.3%)and right bundle branch pattern (26.6%) were the common electrocardiographic findings in our patients. DCRV is commonly associated with other anomalies. Ventricular septal defect (VSD) was the commonest associated anomaly, which was seen in 80% of our patients. Mean gradient cross the anomalous muscle bundle was 67.5 mmHg. Three of our patients (20%) had no associated anomaly, which is very rare in DCRV. Eight patients underwent surgical correction with significant reduction in gradients in all and no perioperative mortality. Median follow up of 8 years showed no adverse outcomes and no progression of gradients.Conclusions: This study describes in detail the clinical profile, echocardiographic and angiographic identification of anomalous muscle bundles in DCRV patients, which will help the young readers in identifying this often missed diagnosis. It highlights the unusual presentation during adulthood with atypical symptoms in DCRV patients with excellent long-term outcomes on follow up.

A Case of Residual Atrial Septal Defect with Left Atrial Isomerism Requiring Reoperation 37 Years after the First Operation / 日本心臓血管外科学会雑誌

A 47-year-old female was admitted to our hospital for management of dyspnea. She had undergone surgery for an atrial septal defect (ASD) at the age of 17. Computed tomography revealed left isomerism, inferior vena cava interruption with azygos continuation and a residual ASD. Intra-operative findings showed that the residual ASD was positioned across the orifice of the hepatic vein. The previous suture line could be identified in the partially-closed atrial septum above the residual defect. Re-closure was performed without difficulties, and the patient's condition was good at discharge. Closure of ASD is a simple and basic procedure in cardiac surgery but care must be taken not to leave a shunt at the lower part of the defect, especially in cases with ASD defects in the lower margin.

Metabolic Syndrome in Adult Congenital Heart Disease

In adult congenital heart disease (ACHD), residua and sequellae after initial repair develop late complications such as cardiac failure, arrhythmias, thrombosis, aortopathy, pulmonary hypertension and others. Acquired lesions with aging such as hypertension, diabetes mellitus, obesity can be negative influence on original cardiovascular disease (CVD). Also, atherosclerosis may pose an additional health problem to ACHD when they grow older and reach the age at which atherosclerosis becomes clinically relevant. In spite of the theoretical risk of atherosclerosis in ACHD due to above mentioned factors, cyanotic ACHDs even after repair are noted to have minimal incidence of coronary artery disease (CAD). Acyanotic ACHD has similar prevalence of CAD as the general population. However, even in cyanotic ACHD, CAD can develop when they have several risk factors for CAD. The prevalence of risk factor is similar between ACHD and the general population. Risk of premature atherosclerotic CVD in ACHD is based, 3 principal mechanisms: lesions with coronary artery abnormalities, obstructive lesions of left ventricle and aorta such as coarctation of the aorta and aortopathy. Coronary artery abnormalities are directly affected or altered surgically, such as arterial switch in transposition patients, may confer greater risk for premature atherosclerotic CAD. Metabolic syndrome is more common among ACHD than in the general population, and possibly increases the incidence of atherosclerotic CAD even in ACHD in future. Thus, ACHD should be screened for metabolic syndrome and eliminating risk factors for atherosclerotic CAD.

Risk factors of prolonged ventilation in adults after atrioventricular septal defect operation / 中国胸心血管外科临床杂志

@#Objective To investigate the risk factors of prolonged postoperative mechanical ventilation for adult patients with atrioventricular septal defect (AVSD). Methods We retrospectively analyzed the clinical data of 76 patients with AVSD aged more than 18 years in our hospital from January 1, 2011 to December 31, 2017. The patients ventilated longer than 24 hours were described as a prolonged ventilation group (n=27) and the others as a normal group (n=49). There were 9 males and 18 females aged 32.22±9.64 years in the prolonged ventilation group, and 16 males and 33 females aged 35.98±11.34 years in the normal group. Perioperative variables between the two groups were compared and selected, and then analyzed by logistic regression analysis. Results The result of univariate analysis showed that there was a statistical difference in weight, preoperative pulmonary artery systolic pressure, duration of cardiopulmonary bypass, the level of postoperative platelet, hemoglobin, blood glucose, lactic acid and serum creatinine, postoperative maximum heart rate and postoperative infection rate between the prolonged ventilated group and the normal group. Multivarable logistic regression showed that preoperative pulmonary artery hypertension (OR=1.056, 95%CI 1.005 to 1.110, P=0.030), prolonged duration of cardiopulmonary bypass (OR=1.036, 95%CI 1.007 to 1.066, P=0.016) and the low postoperative hemoglobin level (OR=0.874, 95%CI 0.786 to 0.973, P=0.014) were the risk factors of prolonged postoperative mechanical ventilation. Conclusion Preoperative pulmonary artery hypertension, long duration of cardiopulmonary bypass and postoperative anaemia are the risk factors associated with prolonged postoperative mechanical ventilation.

Application of bidirectional Glenn procedure in adult congenital heart disease / 中国胸心血管外科临床杂志

@#Objective    To analyze the feasibility of bidirectional Glenn procedure (BDG) in treatment of adult congenital heart disease (ACHD). Methods    From December 2004 to December 2015, 42 ACHD patients received BDG in our hospital. There were 23 males and 19 females with a mean age of 24.6±8.5 years (range: 18 to 49 years). There were functional single ventricle (FSV) in 14 patients, Ebstein’s anomaly in 11, corrected transposition of great arteries in 7, transposition of great arteries in 5, double outlet of right ventricle in 3 and tricuspid atresia in 2. Twenty patients suffered moderate or severe atrioventricular valve regurgitation (AVVR). Half of the patients were operated upon with cardiopulmonary bypass (CPB) and the others with off-pump coronary artery bypass grafting (OPCABG). Thirty-four patients underwent unilateral BDG shunt and eight bilateral BDG shunts. Concomitant procedures included correction of Ebstein’s anomaly (7 patients), atrioventricular valve replacement (7), atrial septostomy (3), ligation of patent ductus arteriosus (3), ligation of major aortopulmonary collateral arteries (2), correction of total anomalous pulmonary venous connection (1) and mitral valve repair (1). Results    The early operative mortality was 9.5% (4/42). FSV and moderate or severe AVVR were risk factors for BDG in ACHD. Early postoperative oxygen saturation increased from 78.8%±11.2% to 89.3%±6.6% (P<0.05). The follow-up time was 6-132 (41.4±33.1) months. There was no death. The heart function improved (2.7±0.5 vs. 1.9±0.4, P<0.05). Conclusion    The BDG shunt can be applied to ACHD. Although the early mortality is relatively high, the middle- and long-term results are satisfactory. The oxygen saturation increases and  the heart function improves. The life quality of patients will also improve. FSV and moderate or severe AVVR are risk factors for BDG in ACHD.

Hipertensión arterial pulmonar en cardiopatías congénitas del adulto: Impacto y resultados Datos del Registro GUTI-GUCH / Pulmonary arterial hypertension in adult congenital heart disease: Impact and outcome Data from the GUTI-GUCH Registry / Hipertensão arterial pulmonar em cardiopatias congênitas no adulto: Impacto e resultados Dados do Registro GUTI-GUCH

Introducción. La hipertensión pulmonar (HP) es una complicación grave de las cardiopatías congénitas del adulto (CCA), su aparición se relaciona con un marcado aumento de la morbimortalidad de estos pacientes. Objetivo. Analizar las características epidemiológicas clínicas y de tratamiento farmacológico en pacientes con hipertensión arterial pulmonar (HAP) y su impacto en la sobrevida. Materiales y métodos. Estudio observacional retrospectivo. Utilizando el Registro GUTI-GUCH, se analizaron datos globales. Se seleccionaron pacientes con diagnóstico de HAP secundaria a cardiopatía congénita (HAPG1) y sus subgrupos. SG1: síndrome de Eisenmenger, SG2: cortocircuitos de izquierda a derecha, SG3: HAP coincidente con cardiopatía congénita, SG4: HAP en posoperatorio. Análisis estadístico: SPSS 20, test del Chi cuadrado, curva de Kaplan Meier contrastada por Log Rank. Resultados. Incorporados 1604 pacientes con edad media de 32,5 años, el 55,4% (889) eran mujeres. Pacientes con cardiopatía de riesgo para HAP (CRHAP): 70,5% (1131); con HP: 4,2% (67) del global y el 5,8% (67) de CRHAP. El 71,6% (48) de los pacientes con HP fueron mujeres, con edad de 41,7 ± 14,4 años y seguimiento de 33± 30,9 meses. Pertenecían al HAPG1 el 91% (61) representando el 3,8% del registro. Los pacientes con HAPG1 se subdividían en: SG1: 47,5% (29); SG2: 26,2% (16); SG3: 5% (3) y SG4: 21,3% (13). El Grupo HAPG1 recibía tratamiento farmacológico, especifico: 78,7% (48), monoterapia: 57,4% (35) y terapia combinada: 21,3% (13). La mortalidad en el seguimiento para los pacientes con HP fue del 17,9% (12) y del 16,4% (10) para HAPG1. Se observaron diferencias estadísticamente significativas para HP: p=0,000; HAPG1: p=0,000; subgrupos de HAPG1: p=0,000. No se encontraron diferencias con el SG1: p=0,184. Conclusiones. La HP y la HAPG1 afectan negativamente la sobrevida de los pacientes con CCA. La mitad de ellos es portador de síndrome de Eisenmenger que junto al SG3 son los de mejor evolución. Se observó una elevada prevalencia de mujeres.

Introduction. Pulmonary hypertension (PH) is a serious complication of adult congenital heart disease (ACHD), its incidence is associated with a marked increase in morbidity and mortality of these patients. Objective. We analyze the epidemiological characteristics clinical and pharmacological treatment in patients with pulmonary arterial hypertension (PAH) and their impact on survival. Materials and methods. Retrospective observational study, global data were analyzed using the GUTI-GUCH Registry. We selected patients with diagnosis of PAH related to congenital heart disease (PAH-CHD) and its subgroups. SG1: Eisenmenger syndrome, SG2: shunts left to right, SG3: PAH-CHD, SG4: postoperative PAH. Statistical analysis: SPSS 20, Chi square test, Kaplan Meier curve contrasted by Log Rank. Results. We included 1604 patients with a mean age of 32.5 years, 55.4% (889) were women. Patients with heart disease risk for PAH (HDRPAH): 70.5% (1131); PH: 4.2% (67) overall and 5.8% (67) of HDRPAH. And 71.6% (48) patients with PH were women, with age 41.7 ± 14.4 years and follow up of 33 ± 30.9 months. The 91% (61) belonged to PAH-CHD group representing 3.8% of registry. PAH-CHD patients were subdivided into: SG1: 47.5% (29); SG2: 26.2% (16); SG3: 5% (3) and SG4: 21.3% (13). The PAH-CHD group received pharmacological treatment, specific: 78.7% (48), monotherapy: 57.4% (35) and combination therapy: 21.3% (13). The follow-up mortality for patients with PH was 17.9% (12) and 16.4% (10) for PAH-CHD. Statistically significant differences were observed for PH: p=0.000; PAH-CHD: p=0.000; PAH-CHD subgroups: p=0.000. No differences were found with SG1: p=0.184. Conclusions. The HP and PAH-CHD negatively affect the survival of patients with ACHD. Half of them are carriers of Eisenmenger syndrome by the SG3 are better evolution. A high prevalence of women was observed.

Introdução. A hipertensão pulmonar (HP) é uma complicação grave das cardiopatias congênitas no adulto (CCA), sua incidência está associada com um aumento significativo na morbidade e mortalidade desses pacientes. Objetivo. Foram analisadas as características epidemiológicas clínicas e de tratamento medicamentoso em pacientes com hipertensão arterial pulmonar (HAP) e seu impacto na sobrevivência. Materiais e métodos. Estudo retrospectivo observacional. Foram analisados dados globais utilizando o Registry GUTI-Guch. Foram selecionados pacientes com diagnóstico de HAP secundária a cardiopatia congênita (HAPG1) e seus subgrupos. SG1: síndrome de Eisenmenger, SG2: desvio de sangue da esquerda à direita, SG3: HAP coincidente com cardiopatia congênita, SG4: HAP pós-operatória. Análise estatística: SPSS 20, teste do qui-quadrado, curva de Kaplan Meier contrastada pela Log Rank. Resultados. Foram incluídos 1604 pacientes com uma idade média de 32,5 anos, o 55,4% (889) eram mulheres. Pacientes com cardiopatia de risco para a HAP (CRHAP): 70,5% (1131); HP: 4,2% (67) do global e 5,8% (67) de CRHAP. O 71,6% (48) dos pacientes com HP eram mulheres, com idade de 41,7 ± 14,4 anos e seguimento de 33 ± 30,9 meses. O 91% (61) pertenciam ao grupo HAPG1, representando o 3,8% do registro. Os pacientes com HAPG1 foram subdivididos em: SG1: 47,5% (29); SG2: 26,2% (16); SG3: 5% (3) e SG4: 21,3% (13). O grupo HAPG1 recebeu tratamento farmacológico, específico: 78,7% (48), monoterapia: 57,4% (35) e uma terapia combinada: 21,3% (13). A mortalidade no seguimento dos pacientes com HP foi de 17,9% (12) e do 16,4% (10) para HAPG1. Foram observadas diferenças estatisticamente significativas para a HP: p=0,000; HAPG1: p=0,000; subgrupos HAPG1: p=0,000. Não foram encontradas diferenças com SG1: p=0,184. Conclusões. A HP e a HAPG1 afetar negativamente a sobrevivência de pacientes com CCA. A metade deles é portadora da síndrome de Eisenmenger e com o SG3 são os de melhor evolução. Observou-se uma alta prevalência de mulheres.

Analysis of early results of adult congenital heart disease underwent surgical correction / 中华胸心血管外科杂志

Objective To summarize 500 cases of surgical experience in restoration of adult congenital heart disease ( ACHD) treatment and early postoperative.Methods During January 2012 to December 2014 in Fuwai Hospital, 500 cases of ACHD treated by operation were chosen to collect the clinical data .We divided the groups according to whether the case was a complex malformation and whether the case had an ICU retention time is more than the 5 days.Results The average age was 35, the average weight was 59 kg.The operation average cardiopulmonary bypass(CPB) time was 102min.The average ICU treatment time was 2 days, the average duration of mechanical ventilation was 23 hours, 3 early deaths occurred.The complex malformation group had younger age and less weight than the simple malformation group , the complex malformation group had longer time of cardiopulmonary bypass time, aortic cross clamping time, mechanical ventilation time and ICU treatment time, had higher rate of complication and blood transfusion peri-operative period than the simple malformation group.(P<0.05) The group of ICU retention time less than 5 days had higher rate of the male proportion, had younger age and less weigh, had longer time of cardiopulmonary bypass time , mechanical ventilation time and ICU treatment time , had higher rate of complication and blood transfusion peri-operative period than the control group(P <0.01).Conclusion Although ACHD patients have long medical history and complicated pathological and physiological changes , when they get proper surgical operation and periopera-tive treatment, they should obtain satisfied effect.Professional medical team or organization service for the ACHD patient is very important and urgent to build.

Ten Years' Experience of Aortic Annulus Ectasia and Ascending-Arch Aortic Aneurysm Cases in Adult Congenital Heart Disease / 日本心臓血管外科学会雑誌

<b>Background</b> : The improvement in surgical results for congenital heart disease has resulted in an increase in the number of adult congenital heart disease (ACHD) cases. Some ACHD patients are known to develop thoracic aortic aneurysm (TAA) at a young age, so we examined TAA in ACHD patients presenting at our institute over a 10-year interval. <b>Methods</b> : From 2002 to 2011, we performed 32 cases of surgery for TAA in ACHD patients. We excluded 5 cases of adult bicuspid aortic valve, 2 of TAA with untreated congenital heart disease (CHD), 1 of Marfan syndrome with CHD, and 9 of coarctation of the aorta (CoA) repair for the same site ; 15 patients were included. <b>Results</b> : The male/female ratio was 13/2, and the age of reoperation was 33.3±10.8 years. The 15 ACHD patients included 5 cases of the tetralogy of Fallot (TOF), 4 of congenital aortic stenosis (AS), 3 of ventricular septal defect (VSD), and 1 of each CoA complex, polysplenia/double outlet right ventricle (DORV), and polysplenia/corrected transposition of the great arteries (cTGA). Twelve cases of root dilatation and 2 of ascending aortic aneurysm were observed and 10 cases were concomitant with moderate to severe aortic regurgitation. Thirteen cases underwent elective surgery and the other two cases were emergency surgeries : a Bentall procedure for type 2 acute aortic dissection of polysplenia/DORV, and a Bentall and right ventricular outflow reconstruction (RVOTR) for ascending aorta/right ventricle rupture due to Konno patch detachment in congenital AS. The 13 elective cases included 11 cases of Bentall procedure, 1 of ascending aorta/hemi arch replacement, and 1 of ascending aorta replacement. Concomitant procedures were 1 case of aortic valve replacement, 1 of mitral valve replacement, 1 of subaortic stenosis release, and 2 of RVOTR. Operation time was 572.8+/-101.4 min, cardiopulmonary bypass time was 295.8+/-100.2 min, and aorta clamp time was 188.1+/-58.8 min. One hospital death was observed in 1 emergency case due to methicillin-resistant <i>Staphylococcus aureus</i> (MRSA) sepsis, but no 30-day mortality was observed. Intensive care unit (ICU) stay was 9.4+/-10.1 days and hospital stay was 34.4+/-18.2 days. <b>Conclusion</b> : The most common ACHD found during TAA surgery in our institute was Tetralogy of Fallot. ACHD had various complications and restrictions for surgery but TAA surgery in ACHD patients was safe and feasible.

Una causa poco frecuente de hipertrabeculación exclusiva del ventrículo derecho: ventrículo derecho no compacto / An unusual cause of right ventricular hypertrabeculation exclusive of the right ventricle: non compaction of right ventricle

La cardiopatía por falta de compactación ventricular es una entidad infrecuente, que generalmente compromete el ventrículo izquierdo ocasionando falla cardíaca, arritmias ventriculares, embolismo periférico y muerte súbita. Hasta el momento, se han descrito muy pocos casos de ventrículo derecho no compacto, por lo cual no hay criterios diagnósticos establecidos y se desconoce como es la evolución clínica y la respuesta al tratamiento de este grupo de pacientes. Se describe el caso de un paciente con hipertrabeculación marcada del ventrículo derecho en quien se descartó un origen estructural de la misma, haciéndose diagnóstico de ventrículo derecho no compacto.

Non compaction cardiomyopathy is a rare entity which usually involves the left ventricle causing heart failure, ventricular arrhythmias, peripheral embolism and sudden death. So far, very few cases of right ventricular non compaction have been described, for which no established diagnostic criteria exist and the clinical course as well as the response to treatment of these patients remain unknown. We describe the case of a patient with marked right ventricular hypertrabeculation in whom a structural origin of it was discarded, whereby the diagnosis of right ventricular non-compactation was made.

Resolución exitosa de la estenosis congénita de las dos ramas de la arteria pulmonar en un adulto mediante el uso de stents / Successful resolution of congenital stenosis of the two branches of the pulmonary artery in an adult using stents

La estenosis de las arterias pulmonares son rara vez diagnosticadas en edad adulta, convirtiéndose su tratamiento en una compleja situación con diferentes posibilidades terapéuticas. Las causas mas frecuentes corresponden a las adquiridas, siendo las mas común la hipertensión pulmonar trombo embólica crónica, quedando reducido numero de casos congénitos asociado principalmente a síndrome genéticos y malformaciones cardiacas asociadas. El tratamiento quirúrgico convencional puede convertirse en un desafío dadas las condiciones anatómicas y funcionales encontradas en estadios avanzados propias del diagnostico tardío y en especial en el caso presentado el cual esta iniciando la sétima década de la vida. La posibilidad de resolución percutánea de estas lesiones cobra importancia a la luz de la amplia utilización de esta en pacientes pediátricos, implementando el uso cada vez mayor de balones con dilatación percutánea y la utilización de stents cada vez más sostificados con buenos resultados. Se presenta nuestro primer caso de resolución percutánea de lesiones coronarias y de arteria pulmonar bilateral en un paciente previamente programado para cirugía de reparación con parche y by pass coronario, el cual evolucionó en formas satisfactoria recobrando su actividad normal a las pocas horas del procedimiento.

Stenosis of the pulmonary arteries is rarely diagnosed in adulthood, turning their treatment in a complex situationwith different therapeutic possibilities. The most frequent causes are acquired, being the most common chronicthromboembolic pulmonary hypertension, remaining a small number of congenital cases mainly associated to geneticsyndromes or complex cardiac malformations. The standard surgical treatment may be a challenge given the anatomicaland functional conditions found themselves in advanced stages of late diagnosis and especially in the case presented,who is beginning the seventh decade of his life. The possibility of percutaneous resolution of these lesions becomesimportant in light of the widespread use of this option in pediatric patients, implementing and increasing its use withpercutaneous dilatation balloons and stents with increasing sophistication and showing good results. We present ourfirst case of percutaneous resolution of coronary lesions and bilateral pulmonary artery stenosis in a patient previouslyscheduled for patch repair surgery and coronary bypass, which evolved in successfully, gaining normal activity within fewhours of the procedure.

Echocardiography in Adult Congenital Heart Diseases

Echocardiography is an important tool in investigation, re-evaluation of remained postoperative defect and follow-up for adult congenital heart disease, even though there are often a problem to obtain optimal echocardiogram. The usual systematic echocardiographic assessments should include special focus on 1) anatomic description of situs, concordance, discordance or valves; 2) chamber sizes and function; 3) valve appearance and function; 4) shunts; 5) pulmonary artery pressure; 6) flow and size of the arch; and 7) drainage of pulmonic veins. The major lesions are presented and echocardiographic features briefly discussed.

Surgical treatment for adult congenital heart disease.

A total of 1,408 adult congenital heart disease patients (age over 13 years) underwent surgery between 1979 and 1995 at Siriraj Hospital, contributing 33.75 percent to the overall number of congenital heart disease cases (4,172) who underwent surgery in that period. This finding is similar to statistics in the West. The ages ranged from 13.3 years to 72 years, with a mean of 24.06 years. The oldest patient was a 72 year-old man with coronary cardiac chamber fistula. The most common diagnoses in descending order were: atrial septal defect (41.05 per cent), patent ductus arteriosus (16.19 per cent), tetralogy of Fallot (14.06 per cent) and ventricular septal defect (10.87 per cent). These four categories covered 82 percent of the cases. The overall surgical mortality was 1.78 per cent, compared with 3 per cent in patients who had undergone surgery during childhood. Only patients with double outlet of the right ventricle and congenital aortic stenosis, or left ventricular outflow tract obstruction, showed increased risk of death (20 per cent), compared with 5.77 per cent in those cases who had undergone surgery during childhood. The conclusions drawn from the study are that surgical treatment for adult congenital heart diseases showed a low average of surgical mortality of less than 2 per cent in almost every category except double outlet of the fight ventricle and congenital aortic stenosis, for which early surgery is recommend.