BACKGROUND Tetralogy of Fallot(TOF)is one of the most common congenital heart defects,and surgery is the primary treatment.There are no precise guidelines on the treatment protocol for tricuspid regurgitation(TR)as a ...BACKGROUND Tetralogy of Fallot(TOF)is one of the most common congenital heart defects,and surgery is the primary treatment.There are no precise guidelines on the treatment protocol for tricuspid regurgitation(TR)as a common complication of TOF repair.The timing for treatment in patients presenting with valve regurgitation after TOF repair is often difficult to determine.Here,we report the first case of sequential treatment of pulmonary and TR using interventional therapy.CASE SUMMARY We present the case of a 52-year-old female patient,who had a history of TOF repair at a young age.A few years later,the patient presented with pulmonary and tricuspid regurgitation.The symptoms persisted and TR worsened following percutaneous pulmonary valve implantation.Preoperative testing revealed that the patient’s disease had advanced to an intermediate to advanced stage and that her general health was precarious.Because open-heart surgery was not an option for the patient,transcatheter tricuspid valve replacement was suggested.This procedure was successful,and the patient recovered fully without any adverse effects.This case report may serve as a useful resource for planning future treatments.CONCLUSION Treatment of both valves should be considered in patients with tricuspid and pulmonary regurgitations following TOF repair.The interventional strategy could be an alternative for patients with poor general health.展开更多
Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow trac...Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients at the level of annulus, STJ & sinus of Valsalva was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). Conclusions: The first important finding of this study is the occurrence of significant aortic root dilatation in 22% of patients after intra-cardiac repair of TOF. Older age at repair, long shunt to repair interval and residual ventricular septal defect are the most common variables associated with aortopathy and aortic regurgitation in such group of patients. The second important finding is the occurrence of aortic root dilatation in 70% of patients before surgical repair of TOF;whereas male sex and TOF with pulmonary atresia appeared to be the most common variables associated with aortopathy and aortic regurgitation in this group of patients.展开更多
Agenesis of pulmonary valve is a rare variant and severe form of Tetralogy of Fallot (ToF). The evolution is usually marked by respiratory and cardiac failure at early age, which needs early surgical correction. Uncor...Agenesis of pulmonary valve is a rare variant and severe form of Tetralogy of Fallot (ToF). The evolution is usually marked by respiratory and cardiac failure at early age, which needs early surgical correction. Uncorrected treatment of Tetralogy of Fallot diagnosed at adult age is infrequent and only few studies have been described. We present here a rare case of a 22 years old patient who presented with dyspnea since childhood. Subsequent investigations allowed diagnosis of treatment of Tetralogy of Fallot with agenesis of the pulmonary valve. Following the assessment, the patient underwent a surgical repair and the recovery was uneventful. The management of treatment of Tetralogy of Fallot with pulmonary valve agenesis in adult period remains complex, requiring different surgical techniques.展开更多
To the Editor:Nowadays,most infants with tetralogy of Fallot(TOF)survive the initial surgical repair;however,they continue to experience residual hemodynamic and physiologic abnormalities in the follow-up.[1]Pulmonary...To the Editor:Nowadays,most infants with tetralogy of Fallot(TOF)survive the initial surgical repair;however,they continue to experience residual hemodynamic and physiologic abnormalities in the follow-up.[1]Pulmonary regurgitation(PR)is still recognized as the most common complication that determines late outcomes.PR may result in right ventricular(RV)dilatation and,ultimately,cardiac dysfunction.Indicators of deterioration of clinical status such as impaired exercise tolerance,ventricular arrhythmia,and sudden cardiac death have all been associated with chronic PR.展开更多
Tetralogy of fallot （TOF） occurs in approximately 1 in 5000 live births and accounts for 12% - 14% of congenital heart disease. Surgical repair was first introduced in the 1950s and there is now a large population o...Tetralogy of fallot （TOF） occurs in approximately 1 in 5000 live births and accounts for 12% - 14% of congenital heart disease. Surgical repair was first introduced in the 1950s and there is now a large population of adults with repaired TOF. Some of them may suffer from significant pulmonary regurgitation （PR）, progressive right ventricle （RV） dilation, RV dysfunction and restrictive right ventricular physiology（RRVP）.展开更多
Background Although most patients with tetralogy of Fallot undergo radical repair during infancy and childhood,patients that remain undiagnosed and untreated until adulthood can still be treated.This study aimed to ev...Background Although most patients with tetralogy of Fallot undergo radical repair during infancy and childhood,patients that remain undiagnosed and untreated until adulthood can still be treated.This study aimed to evaluate longterm outcomes of adult patients with tetralogy of Fallot who were treated surgically,and to determine the predictors of postoperative pulmonary regurgitation.Methods Fifty-six adult patients underwent complete surgical repair.Forty-three patients （76.8%） required a transannular patch.Systolic,diastolic,and mean pressure in the main pulmonary artery were measured after repair.Results The early mortality rate was 3.6%.The 16-year survival rate was （84.4±11.5）%.Late echocardiography revealed 41 patients with transannular patch who had pulmonary regurgitation,consisting of mild pulmonary regurgitation in 28 patients,moderate in eight,and severe regurgitation in five patients.In addition,there was right ventricular outflow tract stenosis in nine patients,moderate/severe tricuspid valve regurgitation in seven,and residual ventricular septal defect in five.Logistic regression analysis demonstrated that the mean pulmonary pressure measured just after repair predicted late pulmonary regurgitation.Conclusions The long-term survival of surgically treated adult patients with tetralogy of Fallot is acceptable.The mean pressure 〉20 mmHg in the main pulmonary artery measured right after surgical repair may be a feasible reference to time the reconstruction of the pulmonary valve.展开更多
Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot （TOF） in developed countries, but rare information for primary correction of adult TOF is...Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot （TOF） in developed countries, but rare information for primary correction of adult TOF is available. The research focusing on the effect of transanular patch （TAP） for primary correction of TOF in adulthood is still absent. Via retrograde analysis of 7-year follow-up, this study was designed to explore the effect of the transanular patch for primary correction in adult TOF on the surgical outcome, postoperative cardiac function and morbidity, as well as to address the management of the complication. Methods A total of 151 consecutive adult patients （age ≥ 18） who underwent primary radical correction of TOF form 2007-2014 were selected and divided into TAP demographic statistic characteristics, and non-TAP groups based on the EACTS database. Results of echocardiography, color-Doppler echocardiography, cardiovascular enhanced contrast computed tomography （CT）, and/or cardiac catheterization; intraoperative information, postoperative results and outcomes were reported. During follow-up, short term was defined within 3 months after discharge, and midterm was defined as 6-12 months after discharge. Results Total postoperative mortality was 5.96% in all the cases, 6.96% in TAP group, and 2.78% （1/36） in non-TAP group. There was no significant difference between two groups. Follow-up period ranged from 3 months to 62 months. Readmission occurred and was followed by medical treatment without re-do surgery in 6 cases （3.97%）. The short term echocardiography demonstrated that pulmonary regurgitation and short term tricuspid regurgitation after discharge in TAP group were more severe （P 〈 0.001）. The short term residual pulmonary stenosis （RVOTO） severity after discharge in TAP group was less severe （P = 0.018）. Midterm echocardiography after discharge demonstrated pulmonary regurgitation and tricuspid regurgitation in TAP group were still more severe （P = 0.003）. The severity of residual pulmonary stenosis in TAP group was less severe （P = 0.044）. Multivariate unconditional logistic regression analysis showed that risk factors for mortality of adult TOF primary correction included： the acquirement of repeated cardiopulmonary bypass, OR = 126.28 （5.17 - 3082.23）, P = 0.003; the application of DHCA, OR = 61.08（2.26 - 1652.51）, P = 0.015; postoperative pulmonary regurgitation, OR = 33.84（2.53 - 452.53）, P = 0.008, long intensive care time, OR = 1.00 （1.00 - 1.01）, P = 0.012. The first three variables were high risk factors. Conclusions Primary radical correction of adult TOF has a good outcome, acceptable morbidity and mortality rates with mid-term surgical outcome in terms of effort tolerance. The acquirement of repeated cardiopulmonary bypass, the application of DHCA and postoperative pulmonary regurgitation are high risk factors of mortality. IS Chin J Cardiol 2015; 16 （2）： 72 - 79]展开更多
文摘BACKGROUND Tetralogy of Fallot(TOF)is one of the most common congenital heart defects,and surgery is the primary treatment.There are no precise guidelines on the treatment protocol for tricuspid regurgitation(TR)as a common complication of TOF repair.The timing for treatment in patients presenting with valve regurgitation after TOF repair is often difficult to determine.Here,we report the first case of sequential treatment of pulmonary and TR using interventional therapy.CASE SUMMARY We present the case of a 52-year-old female patient,who had a history of TOF repair at a young age.A few years later,the patient presented with pulmonary and tricuspid regurgitation.The symptoms persisted and TR worsened following percutaneous pulmonary valve implantation.Preoperative testing revealed that the patient’s disease had advanced to an intermediate to advanced stage and that her general health was precarious.Because open-heart surgery was not an option for the patient,transcatheter tricuspid valve replacement was suggested.This procedure was successful,and the patient recovered fully without any adverse effects.This case report may serve as a useful resource for planning future treatments.CONCLUSION Treatment of both valves should be considered in patients with tricuspid and pulmonary regurgitations following TOF repair.The interventional strategy could be an alternative for patients with poor general health.
文摘Aortic root pathology has been described in patients with Tetralogy of Fallot, although the most common reason for repeat surgery in the adult after TOF repair relates to problems in the right ventricular outflow tract, the aortic root is often forgotten. Objective: We sought to determine those patients with known Fallot tetrallogy at risk for progressive dilatation of the thoracic aorta and explore the common predictors present in this patient group. Methods and Results: A multicenter observational study which enrolled 100 patients (50 surgically repaired and 50 before surgical repair of TOF) with standardized reassessment of echocardiographic parameters and multislice CT angiography of the heart and great vessels data. The data were reviewed and analyzed according to the demographic, morphological, surgical and clinical details. We used standard nomograms and Z score for aortic root dimensions at the level of aortic annulus, sino-tubular junction and sinus of Valsalva based on body surface area. For surgically repaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). For unrepaired patients, all the measured diameters across aortic annulus, STJ & sinus of Valsalva were larger in the dilated unrepaired group with mean & median of 24.63 (3.99) & 25 (15 - 35), 27.2 (4.26) & 27 (17 - 40), 35.97 (4.59) & 36 (24 - 45) mm respectively compared to a mean & median of 13.2 (2.62) & 13 (9 - 17), 14.53 (2.90) & 14 (10 - 19), 20.53 (3.40) & 21 (14 - 25) mm respectively in the not dilated unrepaired group with significant statistical difference (p value < 0.0001). Also Z score among unrepaired dilated TOF patients at the level of annulus, STJ & sinus of Valsalva was larger in comparison to the non dilated unrepaired group with significant statistical difference (p value < 0.0001). Conclusions: The first important finding of this study is the occurrence of significant aortic root dilatation in 22% of patients after intra-cardiac repair of TOF. Older age at repair, long shunt to repair interval and residual ventricular septal defect are the most common variables associated with aortopathy and aortic regurgitation in such group of patients. The second important finding is the occurrence of aortic root dilatation in 70% of patients before surgical repair of TOF;whereas male sex and TOF with pulmonary atresia appeared to be the most common variables associated with aortopathy and aortic regurgitation in this group of patients.
文摘Agenesis of pulmonary valve is a rare variant and severe form of Tetralogy of Fallot (ToF). The evolution is usually marked by respiratory and cardiac failure at early age, which needs early surgical correction. Uncorrected treatment of Tetralogy of Fallot diagnosed at adult age is infrequent and only few studies have been described. We present here a rare case of a 22 years old patient who presented with dyspnea since childhood. Subsequent investigations allowed diagnosis of treatment of Tetralogy of Fallot with agenesis of the pulmonary valve. Following the assessment, the patient underwent a surgical repair and the recovery was uneventful. The management of treatment of Tetralogy of Fallot with pulmonary valve agenesis in adult period remains complex, requiring different surgical techniques.
文摘To the Editor:Nowadays,most infants with tetralogy of Fallot(TOF)survive the initial surgical repair;however,they continue to experience residual hemodynamic and physiologic abnormalities in the follow-up.[1]Pulmonary regurgitation(PR)is still recognized as the most common complication that determines late outcomes.PR may result in right ventricular(RV)dilatation and,ultimately,cardiac dysfunction.Indicators of deterioration of clinical status such as impaired exercise tolerance,ventricular arrhythmia,and sudden cardiac death have all been associated with chronic PR.
文摘Tetralogy of fallot （TOF） occurs in approximately 1 in 5000 live births and accounts for 12% - 14% of congenital heart disease. Surgical repair was first introduced in the 1950s and there is now a large population of adults with repaired TOF. Some of them may suffer from significant pulmonary regurgitation （PR）, progressive right ventricle （RV） dilation, RV dysfunction and restrictive right ventricular physiology（RRVP）.
基金This study was supported by a grant from the Natural Science Foundation ofNingbo, Zhejiang （No. 2011A610036）.
文摘Background Although most patients with tetralogy of Fallot undergo radical repair during infancy and childhood,patients that remain undiagnosed and untreated until adulthood can still be treated.This study aimed to evaluate longterm outcomes of adult patients with tetralogy of Fallot who were treated surgically,and to determine the predictors of postoperative pulmonary regurgitation.Methods Fifty-six adult patients underwent complete surgical repair.Forty-three patients （76.8%） required a transannular patch.Systolic,diastolic,and mean pressure in the main pulmonary artery were measured after repair.Results The early mortality rate was 3.6%.The 16-year survival rate was （84.4±11.5）%.Late echocardiography revealed 41 patients with transannular patch who had pulmonary regurgitation,consisting of mild pulmonary regurgitation in 28 patients,moderate in eight,and severe regurgitation in five patients.In addition,there was right ventricular outflow tract stenosis in nine patients,moderate/severe tricuspid valve regurgitation in seven,and residual ventricular septal defect in five.Logistic regression analysis demonstrated that the mean pulmonary pressure measured just after repair predicted late pulmonary regurgitation.Conclusions The long-term survival of surgically treated adult patients with tetralogy of Fallot is acceptable.The mean pressure 〉20 mmHg in the main pulmonary artery measured right after surgical repair may be a feasible reference to time the reconstruction of the pulmonary valve.
基金supported by The Twelfth National Five-Year Plan(No.2011BAI11B22)
文摘Baekground Although a lot of studies have been performed on the long term outcome in adults with repaired tetralogy of Fallot （TOF） in developed countries, but rare information for primary correction of adult TOF is available. The research focusing on the effect of transanular patch （TAP） for primary correction of TOF in adulthood is still absent. Via retrograde analysis of 7-year follow-up, this study was designed to explore the effect of the transanular patch for primary correction in adult TOF on the surgical outcome, postoperative cardiac function and morbidity, as well as to address the management of the complication. Methods A total of 151 consecutive adult patients （age ≥ 18） who underwent primary radical correction of TOF form 2007-2014 were selected and divided into TAP demographic statistic characteristics, and non-TAP groups based on the EACTS database. Results of echocardiography, color-Doppler echocardiography, cardiovascular enhanced contrast computed tomography （CT）, and/or cardiac catheterization; intraoperative information, postoperative results and outcomes were reported. During follow-up, short term was defined within 3 months after discharge, and midterm was defined as 6-12 months after discharge. Results Total postoperative mortality was 5.96% in all the cases, 6.96% in TAP group, and 2.78% （1/36） in non-TAP group. There was no significant difference between two groups. Follow-up period ranged from 3 months to 62 months. Readmission occurred and was followed by medical treatment without re-do surgery in 6 cases （3.97%）. The short term echocardiography demonstrated that pulmonary regurgitation and short term tricuspid regurgitation after discharge in TAP group were more severe （P 〈 0.001）. The short term residual pulmonary stenosis （RVOTO） severity after discharge in TAP group was less severe （P = 0.018）. Midterm echocardiography after discharge demonstrated pulmonary regurgitation and tricuspid regurgitation in TAP group were still more severe （P = 0.003）. The severity of residual pulmonary stenosis in TAP group was less severe （P = 0.044）. Multivariate unconditional logistic regression analysis showed that risk factors for mortality of adult TOF primary correction included： the acquirement of repeated cardiopulmonary bypass, OR = 126.28 （5.17 - 3082.23）, P = 0.003; the application of DHCA, OR = 61.08（2.26 - 1652.51）, P = 0.015; postoperative pulmonary regurgitation, OR = 33.84（2.53 - 452.53）, P = 0.008, long intensive care time, OR = 1.00 （1.00 - 1.01）, P = 0.012. The first three variables were high risk factors. Conclusions Primary radical correction of adult TOF has a good outcome, acceptable morbidity and mortality rates with mid-term surgical outcome in terms of effort tolerance. The acquirement of repeated cardiopulmonary bypass, the application of DHCA and postoperative pulmonary regurgitation are high risk factors of mortality. IS Chin J Cardiol 2015; 16 （2）： 72 - 79]
