Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sin...Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinusnode dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changingthe pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II,and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart ratecould be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioningLV.展开更多
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
Objectives: The purpose was to show how important is to operate on the Senegalese African children presenting with Tetralogy of Fallot (TOF) regardless of their age. Methods: It is a retrospective, descriptive analysi...Objectives: The purpose was to show how important is to operate on the Senegalese African children presenting with Tetralogy of Fallot (TOF) regardless of their age. Methods: It is a retrospective, descriptive analysis of data from the Department of Pediatric Cardiology, University Hospital of Tuebingen’s data base which was searched for all cases of TOF in foreign patients younger than 15 years between 05/2004-10/2016. Results: 16 children from Senegal with TOF were referred for treatment. Mean age of patients was 7.0 years (range 0.9 - 14.8 years). Primary corrective surgery was performed in 13/16 patients. A primary shunt procedure was required in 3/16 patients. All patients were discharged in good condition with a median 13 postoperative days. We did not register any deaths. All became asymptomatic with pulse oximetry oxygen saturations greater than 95%. The right ventricular outflow tract (RVOT) gradient was less than 30 mmHg in all patients and 2 patients had moderate pulmonary valvular insufficiency. Conclusions: It was still necessary to operate Senegalese children presenting with TOF irrespective of their age.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
Since the first identification of Tetralogy of Fallot in 1671,consisting of a combination of anatomical defects including biventricular origin of the aorta,maligned ventricular septal defect,overriding aorta,and narro...Since the first identification of Tetralogy of Fallot in 1671,consisting of a combination of anatomical defects including biventricular origin of the aorta,maligned ventricular septal defect,overriding aorta,and narrowing or atresia of the pulmonary outflow tract.The first successful operation consisted of a shunt between the left subclavian artery and pulmonary artery.Following this palliative procedure,complete repair is performed once the patient reaches indicative criteria.Since the first attempts at surgical palliation and repair,techniques and outcomes have improved drastically.Definitive repair of Tetralogy of Fallot consists of amulti-patch closure of anyVentricular SeptalDefect along with clearance of any muscular obstructions of the Right Ventricular Outflow Tract and reconstruction of the outflow tract.Current results of Tetralogy of Fallot palliation yield excellent long and short-term results with 5-year freedom from reintervention of 90%.The iterative improvement of repair techniques has greatly reduced intraoperative and postoperative complications.Future innovations such as increased use of percutaneous repair methods and additional data on the benefits of primary repair as opposed to staged palliation will continue to improve patient outcomes.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
Objective:Annulus-sparing(AS)repair for tetralogy of Fallot(TOF)with a dysplastic pulmonary valve annulus(PVA)is a challenging procedure and is controversial.This study aimed to assess the feasibility and surgical eff...Objective:Annulus-sparing(AS)repair for tetralogy of Fallot(TOF)with a dysplastic pulmonary valve annulus(PVA)is a challenging procedure and is controversial.This study aimed to assess the feasibility and surgical effect of AS repair versus transannular patch enlargement(TAPE)repair,especially in individuals with dysplastic pulmonary valves.Methods:This retrospective cohort study included 375 pediatric patients with a primary diagnosis of TOF in the Center for Pediatric Cardiac Surgery of Fuwai hospital from January 2014 to June 2017.Among them,60 consecutive and nonselective patients underwent 1-stage repair of TOF with aggressive PVA-preserving strategies performed by a single surgeon were enrolled in AS cohort.In AS cohort,patients were divided into AS,PVA z-score≥−2 group(33 patients)and AS,PVA z-score<−2 group(27 patients).During the same period,315 patients underwent TAPE repair by other surgeons were enrolled as TAPE cohort,of these,87 patients with PVA z-score≥−2 were excluded.From the 228 patients in the TAPE group,27 cases were selected as TAPE,PVA z-score<−2 group according to the propensity score and 1:1 ratio with AS,PVA z-score<−2 group.The primary outcome was a composite of reintervention,significant pulmonary regurgitation,and significant annular peak gradient(APG).Kaplan-Meier curve was plotted to show the survival rate of severe pulmonary regurgitation.Results:One death occurred after the TAPE operation in TAPE group,and 1 patient in the AS z≥−2 group needed reintervention with a balloon.After a median follow-up of(30.3±11.6)months,compared with AS z≥−2 group,there was no difference in the technical performance score for severe pulmonary stenosis(APGs>20 mmHg)in the AS z<−2 group.Compared with TAPE,AS repair was often accompanied by a postoperative APGs over 20 mmHg(P=0.001).More patients underwent TAPE suffered from moderate or severe pulmonary regurgitation than those who received AS repair(20(74.1%)vs.7(26.0%),P<0.001).AS repair was associated with a shorter duration of mechanical ventilation(20 vs.29 hours,P=0.039),faster discharge from the intensive care unit(2.0 vs.4.0 days,P=0.022)and shorter postoperative hospitalization(8.0 vs.11.0 days,P=0.008)compared with TAPE.Conclusions:APG demonstrated an upward trend in the TAPE group and a downtrend in the AS group after discharge from hospital.AS repair had an acceptable surgical effect in TOF patients,even in those with a dysplastic PVA.A higher APG remained upon hospital discharge in dysplastic patients with AS,but a downward trend was observed over time.展开更多
文摘Appropriate heart rate in a failing pulmonary ventricle remains unknown, particularly in congenital heart diseasewith unique hemodynamics. A 71-year-old male with repaired tetralogy of Fallot and a pacemaker for a sinusnode dysfunction suffered from heart failure symptoms with preserved left ventricular function. Simply changingthe pacemaker’s lower rate from 60 to 75 bpm, New York Heart Association classification improved from III to II,and hemodynamic parameters drastically improved. We regarded this case as informative. Appropriate heart ratecould be higher in congenital patients with failing right and non-failing left ventricles than in adults with malfunctioningLV.
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Objectives: The purpose was to show how important is to operate on the Senegalese African children presenting with Tetralogy of Fallot (TOF) regardless of their age. Methods: It is a retrospective, descriptive analysis of data from the Department of Pediatric Cardiology, University Hospital of Tuebingen’s data base which was searched for all cases of TOF in foreign patients younger than 15 years between 05/2004-10/2016. Results: 16 children from Senegal with TOF were referred for treatment. Mean age of patients was 7.0 years (range 0.9 - 14.8 years). Primary corrective surgery was performed in 13/16 patients. A primary shunt procedure was required in 3/16 patients. All patients were discharged in good condition with a median 13 postoperative days. We did not register any deaths. All became asymptomatic with pulse oximetry oxygen saturations greater than 95%. The right ventricular outflow tract (RVOT) gradient was less than 30 mmHg in all patients and 2 patients had moderate pulmonary valvular insufficiency. Conclusions: It was still necessary to operate Senegalese children presenting with TOF irrespective of their age.
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.
基金Taufiek Konrad Rajab’s research is supported by the National Institutes of Health/National Heart,Lung,and Blood Institute grant R41 HL169059the American Association for Thoracic Surgery,the Brett Boyer Foundation,the Saving tiny Heart Society+1 种基金the Emerson Rose Heart Foundation,philanthropy from Senator Campbell and the Arkansas Children’s Research Institutesupported with funding by Children’s Mercy Kansas City Ward Family Heart Center.
文摘Since the first identification of Tetralogy of Fallot in 1671,consisting of a combination of anatomical defects including biventricular origin of the aorta,maligned ventricular septal defect,overriding aorta,and narrowing or atresia of the pulmonary outflow tract.The first successful operation consisted of a shunt between the left subclavian artery and pulmonary artery.Following this palliative procedure,complete repair is performed once the patient reaches indicative criteria.Since the first attempts at surgical palliation and repair,techniques and outcomes have improved drastically.Definitive repair of Tetralogy of Fallot consists of amulti-patch closure of anyVentricular SeptalDefect along with clearance of any muscular obstructions of the Right Ventricular Outflow Tract and reconstruction of the outflow tract.Current results of Tetralogy of Fallot palliation yield excellent long and short-term results with 5-year freedom from reintervention of 90%.The iterative improvement of repair techniques has greatly reduced intraoperative and postoperative complications.Future innovations such as increased use of percutaneous repair methods and additional data on the benefits of primary repair as opposed to staged palliation will continue to improve patient outcomes.
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supported by the Central Public-interest Scientific Institution Basal Research Fund(2019XK320050)Central University Basic Research Fund(APL20100410010302004)Yunnan Provincial Cardiovascular Disease Clinical Medical Center Project(FZX2019-06-01).
文摘Objective:Annulus-sparing(AS)repair for tetralogy of Fallot(TOF)with a dysplastic pulmonary valve annulus(PVA)is a challenging procedure and is controversial.This study aimed to assess the feasibility and surgical effect of AS repair versus transannular patch enlargement(TAPE)repair,especially in individuals with dysplastic pulmonary valves.Methods:This retrospective cohort study included 375 pediatric patients with a primary diagnosis of TOF in the Center for Pediatric Cardiac Surgery of Fuwai hospital from January 2014 to June 2017.Among them,60 consecutive and nonselective patients underwent 1-stage repair of TOF with aggressive PVA-preserving strategies performed by a single surgeon were enrolled in AS cohort.In AS cohort,patients were divided into AS,PVA z-score≥−2 group(33 patients)and AS,PVA z-score<−2 group(27 patients).During the same period,315 patients underwent TAPE repair by other surgeons were enrolled as TAPE cohort,of these,87 patients with PVA z-score≥−2 were excluded.From the 228 patients in the TAPE group,27 cases were selected as TAPE,PVA z-score<−2 group according to the propensity score and 1:1 ratio with AS,PVA z-score<−2 group.The primary outcome was a composite of reintervention,significant pulmonary regurgitation,and significant annular peak gradient(APG).Kaplan-Meier curve was plotted to show the survival rate of severe pulmonary regurgitation.Results:One death occurred after the TAPE operation in TAPE group,and 1 patient in the AS z≥−2 group needed reintervention with a balloon.After a median follow-up of(30.3±11.6)months,compared with AS z≥−2 group,there was no difference in the technical performance score for severe pulmonary stenosis(APGs>20 mmHg)in the AS z<−2 group.Compared with TAPE,AS repair was often accompanied by a postoperative APGs over 20 mmHg(P=0.001).More patients underwent TAPE suffered from moderate or severe pulmonary regurgitation than those who received AS repair(20(74.1%)vs.7(26.0%),P<0.001).AS repair was associated with a shorter duration of mechanical ventilation(20 vs.29 hours,P=0.039),faster discharge from the intensive care unit(2.0 vs.4.0 days,P=0.022)and shorter postoperative hospitalization(8.0 vs.11.0 days,P=0.008)compared with TAPE.Conclusions:APG demonstrated an upward trend in the TAPE group and a downtrend in the AS group after discharge from hospital.AS repair had an acceptable surgical effect in TOF patients,even in those with a dysplastic PVA.A higher APG remained upon hospital discharge in dysplastic patients with AS,but a downward trend was observed over time.
