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The Thoracic and Cardiovascular Surgeon Dec 2022The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF).
BACKGROUND
The aim of this study was to evaluate the long-term outcome and freedom from pulmonary valve replacement (PVR) after initial repair of tetralogy of Fallot (TOF).
PATIENTS AND METHODS
The cohort of 306 patients treated between 1980 and 2017 was divided into anatomical subgroups according to the diagnosis of TOF-pulmonary stenosis, TOF-pulmonary atresia and TOF-double outlet right ventricle. Patients were treated with transannular patch (TAP), valve sparing repair (VSR), or conduits from the right ventricle to the pulmonary arteries (RVPA conduits).
RESULTS
There were 21 deaths (6.9%), 14 being hospital deaths (4.6%) after primary correction and four deaths (1.3%) occurred after PVR. One patient died after a non-cardiac operation (0.3%). There were two late deaths (0.7%). During the past 12 years no early mortality has been observed. Ninety-one patients (30.4%) received PVR after a median of 12.1 ± 7.0 years with an early mortality of 4.4% ( = 4) and no late mortality. A significant difference in freedom from reoperation after TAP, VSR, and RVPA-conduits could be identified. Multivariate analysis displayed transannular repair ( = 0.016), primary palliation ( <0.001), the presence of major aortopulmonary collateral arteries (MAPCA; = 0.023), and pulmonary valve -scores < - 4.0 ( = 0.040) as significant risk factors for PVR.
CONCLUSION
TOF repair has a beneficial long-term prognosis with low morbidity and mortality. Pulmonary valve -scores < - 4.0, transannular repair, and presence of MAPCAs are associated with earlier PVR. Non-VSRs and TOF-pulmonary atresia lead to earlier reoperation but have no negative impact on survival.
Topics: Cardiac Surgical Procedures; Humans; Infant; Pulmonary Atresia; Pulmonary Valve; Reoperation; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome
PubMed: 35752174
DOI: 10.1055/s-0042-1749098 -
CJC Pediatric and Congenital Heart... Dec 2023Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch... (Review)
Review
Tetralogy of Fallot with pulmonary stenosis has a diverse clinical spectrum with the degree of right ventricular outflow tract obstruction (RVOTO) and size of the branch pulmonary arteries driving clinical management. Optimal surgical management involves consideration of patient clinical status and degree and location (subvalvar, valvar, and supravalvar) of RVOTO. Timing of repair requires multidisciplinary decision-making and complete surgical repair with relief of RVOTO by either transannular patch or valve sparing repair techniques. The central goals of contemporary surgical management of tetralogy of Fallot incorporate maximizing survival, minimizing reintervention, and preserving right ventricular function across the lifespan.
PubMed: 38161666
DOI: 10.1016/j.cjcpc.2023.09.003 -
Indian Heart Journal 2024There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of... (Review)
Review
There is conclusive evidence for relief of pulmonary valve obstruction immediately after balloon pulmonary valvuloplasty (BPV) and at follow-up. Development of infundibular obstruction is seen in more severe PS cases and in older subjects. Reappearance of PS was observed in approximately 10 % of patients following BPV. The reasons for recurrence were found to be balloon/annulus ratio less than 1.2 and immediate post-BPV pulmonary valve peak gradients greater than 30 mmHg. Recurrent stenosis is successfully addressed by repeating BPV with lager balloons than used initially. Long-term results revealed continue relief of obstruction, but with development of pulmonary insufficiency, some patients requiring replacement of the pulmonary valve. It was concluded that BPV is the treatment of choice in the management valvar PS and that balloon/annuls ratio used for BPV should be lowered to 1.2 to 1.25. It was also suggested that strategies should be developed to prevent/reduce pulmonary insufficiency at long-term follow-up.
Topics: Humans; Balloon Valvuloplasty; Pulmonary Valve Stenosis; Pulmonary Valve; Treatment Outcome
PubMed: 38147974
DOI: 10.1016/j.ihj.2023.12.007 -
Frontiers in Genetics 2022Common cardiac abnormalities in Noonan syndrome (NS) include congenital heart diseases (CHD), pulmonary valve stenosis and hypertrophic cardiomyopathy (HCM). Molecular...
Common cardiac abnormalities in Noonan syndrome (NS) include congenital heart diseases (CHD), pulmonary valve stenosis and hypertrophic cardiomyopathy (HCM). Molecular diagnoses are enabling earlier and more precise diagnosis of patients who have a subtle or atypical presentation. The aims of this study were to investigate genotype-phenotype associations with respect to Noonan syndrome (NS)-associated cardiac abnormalities and catheter or surgery-based interventions conditions. From January 2019 to December 2021, 22 children with a confirmed molecular diagnosis of NS combined with cardiovascular abnormalities were consecutively enrolled into the current study. A comprehensive review was carried out of echocardiography and electrocardiogram results, second-generation whole-exome sequencing results and catheter or surgery-based interventions conditions. The main manifestations of electrocardiogram abnormalities were QTc prolongation, abnormal Q wave in the precordial lead and limb lead, right ventricular hypertrophy and left or right deviation of the electrical axis. The most commonly detected abnormality was pulmonary valve dysplasia with stenosis, seen in 15 (68.2%) patients, followed by atrial septal defect in 11 (50%) patients. Seven genes (, , , , , , and ) were found to contain disease-associated variants The most commonly observed genetic mutations were (27%) and (27%) Each genotype was associated with specific phenotypic findings. , , , and had common echocardiography features characterized by pulmonary valve stenosis, while was characterized by HCM. Interestingly, patients with mutations were not only characterized by HCM, but also by pulmonary valve stenosis. In the cohort there was only one patient carrying a mutation characterized by left ventricle globose dilation. Ten cases underwent catheter or surgery-based interventions. All the operations had immediate results and high success rates. However, some of the cases had adverse outcomes during extended follow-up. Based on the genotype-phenotype associations observed during follow-up, and genotypes seem to be poor prognostic factors, and multiple interventions may be required for NS patients with severe pulmonary stenosis or myectomy for HCM. The identification of causal genes in NS patients has enabled the evaluation of genotype-cardiac phenotype relationships and prognosis of the disease. This may be beneficial for the development of therapeutic approaches.
PubMed: 35770001
DOI: 10.3389/fgene.2022.915129 -
Archives of Cardiovascular Diseases Feb 2020Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the... (Review)
Review
Fetal cardiac intervention (FCI) is a novel and evolving technique that allows for in utero treatment of a subset of congenital heart disease. This review describes the rationale, selection criteria, technical features, and current outcomes for the three most commonly performed FCI: fetal aortic stenosis with evolving hypoplastic left heart syndrome (HLHS); HLHS with intact or restrictive atrial septum; and pulmonary atresia with intact ventricular septum, with concern for worsening right ventricular (RV) hypoplasia.
Topics: Aortic Valve Stenosis; Cardiac Catheterization; Cardiac Surgical Procedures; Echocardiography; Female; Fetal Heart; Fetal Therapies; Gestational Age; Heart Defects, Congenital; Humans; Hypoplastic Left Heart Syndrome; Pregnancy; Pulmonary Atresia; Risk Factors; Treatment Outcome; Ultrasonography, Prenatal
PubMed: 32113817
DOI: 10.1016/j.acvd.2019.06.007 -
Pediatric Investigation Dec 2022Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the... (Review)
Review
Surgical repair of a variety of congenital heart diseases involves repair of the right ventricular outflow tract (RVOT) with valved or non-valved conduit to connect the right ventricle (RV) to the pulmonary artery (PA) or just patch enlargement of the native RVOT. With time, this RV-PA conduit will degenerate with deterioration of function, either causing pulmonary stenosis or pulmonary regurgitation. This RVOT dysfunction may result in RV dilation, RV dysfunction, and eventual RV failure and arrhythmias. Multiple surgical pulmonary valve replacement (PVR) is often required throughout the patient's lifetime. Patients are subjected to increased risks with each additional cardiac operation. Transcatheter PVR (TPVR) has been developed over the past two decades as a valuable non-surgical alternative to restore the RVOT and RV function, and hence reduce patients' lifetime risks related to surgery. This article will discuss the long-term results of TPVR which are demonstrated to be comparable to surgical results and the latest development of large pulmonary valves which will allow TPVR to be performed on native or larger RVOT.
PubMed: 36582274
DOI: 10.1002/ped4.12359 -
Frontiers in Cardiovascular Medicine 2022
PubMed: 35924216
DOI: 10.3389/fcvm.2022.970515 -
Medicine and Pharmacy Reports Jan 2020Twin-to-twin transfusion syndrome (TTTS) is the consequence of vascular anastomoses of the shared placenta of monochorionic twin pregnancies. Both circulating inter-twin... (Review)
Review
Twin-to-twin transfusion syndrome (TTTS) is the consequence of vascular anastomoses of the shared placenta of monochorionic twin pregnancies. Both circulating inter-twin blood flow and vasoactive mediators imbalance cause hypovolemia in the donor and hypervolemia in the recipient fetus. If left untreated, TTTS has a high perinatal mortality rate and adverse long-term outcomes mainly cardiovascular and neurological. The recipient has cardiovascular changes including atrioventricular valve regurgitation, diastolic dysfunction and pulmonary stenosis/atresia. The maladaptive response to vascular changes determines a constant decreased blood flow in the donor that permanently modifies the arterial structure leading to postnatal alterations in the vascular system. Fetoscopic LASER surgery of placental vascular anastomoses may disrupt the underlying pathophysiology and improves cardiovascular function with normalization of systolic and diastolic function within weeks after treatment. The impact of cardiovascular changes is relevant for the safety of the management of a TTTS case. The improvement of the perinatal survival after intrauterine surgery leads to viable infants with the longer-term sequelae. Therefore accurate quantification of cardiovascular involvement is essential for clinicians for pregnancy management but also for patient counseling about the potential treatment options the outcome.
PubMed: 32133441
DOI: 10.15386/mpr-1481