Humans ; DiGeorge Syndrome/diagnostic imaging* ; Tetralogy of Fallot/surgery* ; Ultrasonography

Objective: To investigate the clinical and electrophysiological features of ventricular tachycardia (VT) in tetralogy of Fallot (TOF) patients post surgical repair (rTOF) and to analyze the therapeutic effect and prognosis of radiofrequency ablation of rTOF-VT. Methods: This is a retrospective study. Consecutive patients with rTOF-VT, who were treated in Fuwai Hospital from January 2015 to March 2020, were enrolled. All the patients underwent right ventricular voltage mapping following routine cardiac electrophysiological examination, followed by linear or homogenizing radiofrequency ablation based on the low-voltage substrate. The clinical features, 3-dimentional electrophysiological substrate mapping, radiofrequency ablation and long-term prognosis of the enrolled patients were analyzed. Acute ablation success was defined as completion of linear or homogenizing ablation or intraoperative evoked VT as destination of the procedure. Patients were followed up at 3 and 6 months post operation and every year thereafter. The endpoints were sudden cardiac death (SCD) and recurrence of ventricular tachycardia. Results: A total of 20 patients with rTOF-VT were enrolled including 14 males with an age of (35.8±11.8) years. The electrocardiogram identified 23 types of ventricular tachycardia, 19 of which were originated from right ventricular inflow tract outlet. The most common clinical manifestations were heart murmur (19 cases, 95%) and syncope (4 cases, 25%). Electroanatomical substrate mapping was performed in 20 patients and evidenced localized or diffuse scar or low-voltage area of right ventricle. Intraoperative electrophysiological tests provoked ventricular tachycardia in 6 patients (30%), including 5 patients with hemodynamics disturbance. The acute success rate of radiofrequency ablation was 95% (19/20). The follow-up time was (31.1±17.7) months and the recurrence rate of ventricular tachycardia was 30% during follow-up period and 5 cases received repeat radiofrequency ablation and there was no recurrent ventricular tachycardia during follow-up post repeat radiofrequency ablation. Conclusions: The voltage substrate mapping under sinus rhythm is a feasible mapping method for rTOF-VT. Linear or flaky radiofrequency ablation of the slow conduction zone is safe and effective treatment strategy, the recurrence rate after the first radiofrequency ablation is still high, and the effectiveness of repeat radiofrequency ablation is satisfactory in this patient cohort.
Adult ; Arrhythmias, Cardiac ; Catheter Ablation ; Electrocardiography ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; Retrospective Studies ; Tachycardia, Ventricular/surgery* ; Tetralogy of Fallot/surgery* ; Treatment Outcome ; Young Adult

Congenital heart disease (CHD) is now more common in adults than in children due to improvements in fetal echo, neonatal and pediatric care, and surgical techniques leading to dramatically increased survivability into adulthood. Adult patients with CHD, regardless of prior cardiac surgery, experience further cardiac problems or therapeutic challenges; therefore, a non-invasive, easily accessible echocardiographic examination is an essential follow-up tool. Among echocardiographic modalities, three-dimensional (3D) echocardiography provides better delineation of spatial relationships in complex cardiac geometries and more accurate volumetric information without geometric assumptions. For atrial septal defects, an en face view of the tissue defect allows better decisions on device closure. For tricuspid valve malformations, an en face view provides diagnostic information that is difficult to obtain from routine 2D tomography. In repaired tetralogy of fallot with pulmonary regurgitation, preoperative 3D echocardiography- based right ventricular volume may be used to determine the timing of a pulmonary valve replacement in conjunction with cardiovascular magnetic imaging. For optimal adult CHD care, 3D echocardiography is an important complement to routine 2D echocardiography.
Adult* ; Child ; Complement System Proteins ; Echocardiography ; Echocardiography, Three-Dimensional* ; Follow-Up Studies ; Heart Defects, Congenital* ; Heart Septal Defects, Atrial ; Humans ; Pulmonary Valve ; Pulmonary Valve Insufficiency ; Tetralogy of Fallot ; Thoracic Surgery ; Tricuspid Valve

Tetralogy of Fallot (TOF) is one of the most common forms of cyanotic congenital heart disease usually managed by serial surgical repairs. The repaired prosthetic valve or conduit is susceptible to life-threatening infection. FDG-PET is an effective alternative to evaluate the source of infection when other examinations are inconclusive. We report an unusual case of an infected pulmonary artery conduit after TOF repair although the echocardiogram was negative for vegetation, which was later confirmed by surgery and pathology. The case highlights the role of FDG-PETas a problem-solving tool for potential endocarditis and cardiac device infection cases after complex cardiac surgery.
Endocarditis ; Heart Defects, Congenital ; Pathology ; Pulmonary Artery ; Tetralogy of Fallot ; Thoracic Surgery

PURPOSE: This study aimed to evaluate and compare the characteristics of infective endocarditis (IE) between children and adults with congenital heart disease (CHD) at a single tertiary care center. METHODS: In this retrospective medical record review, we extracted the demographic characteristics, diagnostic variables, and outcomes of patients diagnosed with IE and CHD between 2000 and 2016. RESULTS: We identified a total of 14 pediatric patients (nine male; median age at diagnosis, 3 years). Of the 14 patients, six had a history of previous open heart surgery, while four had undergone tetralogy of Fallot repair, with transannular patch or Rastelli procedure. Among the 10 children with positive blood cultures, the most common isolated organism was Staphylococcus spp. (8/10, 80%). Eleven adult patients had IE and CHD. Among the adult patients, only four were diagnosed with CHD before IE, and ventricular septal defect was the most common CHD. The most common isolated organism was Streptococcus spp. (6/11, 55%). Compared with adult patients, pediatric patients had a higher incidence of previously diagnosed CHD (P=0.001), with Staphylococcus spp. as the causative organism (P =0.027). The median duration between the onset of symptoms and diagnosis of IE was 9 days in children and 42 days in adults (P=0.012). CONCLUSIONS: Significant differences with regard to the diagnosis and progress of IE were observed between children and adults. Ageadjusted and systematic reassessment may be necessary for the diagnosis and management of IE.
Adult* ; Arterial Switch Operation ; Child* ; Diagnosis ; Endocarditis* ; Heart Defects, Congenital* ; Heart Septal Defects, Ventricular ; Humans ; Incidence ; Male ; Medical Records ; Retrospective Studies ; Staphylococcus ; Streptococcus ; Tertiary Care Centers* ; Tertiary Healthcare* ; Tetralogy of Fallot ; Thoracic Surgery

Recently, surgical outcomes of repair of tetralogy of Fallot (TOF) have improved. For patients with TOF older than 3 months, primary repair has been advocated regardless of symptoms. However, a surgical approach to symptomatic TOF in neonates or very young infants remains elusive. Traditionally, there have been two surgical options for these patients: primary repair versus an initial aortopulmonary shunt followed by repair. Early primary repair provides several advantages, including avoidance of shunt-related complications, early relief of hypoxia, promotion of normal lung development, avoidance of ventricular hypertrophy and fibrosis, and psychological comfort to the family. Because of advances in cardiopulmonary bypass techniques and accumulated experience in neonatal cardiac surgery, primary repair in neonates with TOF has been performed with excellent early outcomes (early mortality<5%), which may be superior to the outcomes of aortopulmonary shunting. A remaining question regarding surgical options is whether shunts can preserve the pulmonary valve annulus for TOF neonates with pulmonary stenosis. Symptomatic neonates and older infants have different anatomies of right ventricular outflow tract (RVOT) obstructions, which in neonates are nearly always caused by a hypoplastic pulmonary valve annulus instead of infundibular obstruction. Therefore, a shunt is less likely to preserve the pulmonary valve annulus than is primary repair. Primary repair of TOF can be performed safely in most symptomatic neonates. Patients who have had primary repair should be closely followed up to evaluate the RVOT pathology and right ventricular function.
Anoxia ; Cardiopulmonary Bypass ; Fibrosis ; Humans ; Hypertrophy ; Infant ; Infant, Newborn* ; Lung ; Pathology ; Pulmonary Atresia* ; Pulmonary Valve ; Pulmonary Valve Stenosis ; Tetralogy of Fallot* ; Thoracic Surgery ; Ventricular Function, Right

BACKGROUNDAlthough 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.

METHODSFifty-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.

RESULTSThe 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.

CONCLUSIONSThe 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.

Adolescent ; Adult ; Female ; Humans ; Logistic Models ; Male ; Middle Aged ; Postoperative Complications ; etiology ; Pulmonary Artery ; physiopathology ; Pulmonary Valve Insufficiency ; etiology ; Survival Rate ; Tetralogy of Fallot ; mortality ; physiopathology ; surgery ; Treatment Outcome

OBJECTIVETo summarize the experience with surgical correction of tetralogy of Fallot in adults over 40 years of age.

METHODSFrom November 1985 to July 2008, 9 male and 11 female patients aged 41-53 years (mean 46.3±3.5 years) underwent total surgical correction for tetralogy of Fallot. Twelve patients had preoperative NYHA class III cardiac function. The common comorbidities included infective endocarditis, cerebral abscess, cerebral infarction, renal dysfunction, and tricuspid insufficiency. Surgical corrections were carried out at the anatomical or physiological level.

RESULTSNineteen patients received right ventriculotomy to relieve right ventricular outflow obstruction and for ventricular septal defect closure, and 1 patient had Fontan operation. Two patients died after the surgery for heart failure and ventricular fibrillation. The average cardiopulmonary bypass time, aortic clamp time, and postoperative ventilation time was 142.9±36.3 min, 89.9±25.1 min, and 72.0±17.5 h, respectively. Postoperative low cardiac output syndrome occurred in 5 cases, septic shock in 1 case, secondary renal failure in 1 case, and bleeding in 2 cases. Echocardiography showed a significant postoperative reduction of the mean right ventricular outflow tract velocity from 4.29±1.36 m/s to 2.13±0.83 m/s (P<0.01); the right ventricular longitudinal dimension exhibited no significant changes postoperatively (57.1±6.7 mm vs 55.1±7.0 mm, P=0.65).

CONCLUSIONSSurgical correction of the tetralogy of Fallot in patients over 40 years is highly risky and requires appropriate management of cardiac failure, careful myocardial protection, and thorough intracardiac lesion correction to decrease surgical complications.

Adult ; Female ; Humans ; Male ; Middle Aged ; Tetralogy of Fallot ; surgery ; Treatment Outcome

We herein report a rare case of subaortic stenosis in association with a previous tetralogy of Fallot (TOF) surgical repair, which was not taken into account as a differential diagnosis. Echocardiography plays a pivotal role in identification of this rare combination. Therefore, echocardiography should be performed periodically during follow-up of patients with surgically corrected TOF. Given the clinical complications that can result from subaortic stenosis (i.e., aortic regurgitation and infective endocarditis), early and aggressive management of this rare combination should be performed.
Adult ; Aortic Valve Insufficiency/etiology ; Discrete Subaortic Stenosis/*complications/surgery/ultrasonography ; Echocardiography ; Female ; Heart Defects, Congenital/*complications/surgery/ultrasonography ; Humans ; Tetralogy of Fallot/*complications/surgery/ultrasonography ; Time Factors

A 10-year-old boy who had previously undergone surgical correction for tetralogy of Fallot was referred for cardiac catheterization because of recurrent symptomatic postoperative heart failure owing to major aortopulmonary collateral arteries (MAPCAs). A successful occlusion of these MAPCAs was achieved percutaneously using the Amplatzer vascular plug and a detachable coil. Transcatheter occlusion of MAPCAs after surgical correction is an effective method for treatment of post-operative heart failure.
Child ; Collateral Circulation ; Heart Failure ; surgery ; Humans ; Male ; Postoperative Period ; Pulmonary Atresia ; surgery ; Tetralogy of Fallot ; surgery