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Hellenic Journal of Cardiology : HJC =... 2022The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter... (Review)
Review
The majority of patients with congenital heart disease (CHD) who have undergone open heart surgery during childhood are possible candidates for additional transcatheter or surgical interventions. One fifth of these conditions usually involve the right ventricular outflow tract (RVOT). Percutaneous pulmonary valve replacement (PPVR) has been widely established as an alternative, less invasive option to surgical pulmonary valve replacement (SPVR). The variability of RVOT anatomy and size, the relative course of the coronary arteries, and the anatomy of the pulmonary artery branches are factors that determine the success of the intervention and the complication rates. Careful and reliable pre-interventional imaging warrants the selection of suitable candidates and minimizes the risk of complications. 2D and 3D fluoroscopy have been extensively used during pre- and peri-interventional assessment. Established imaging techniques such as cardiovascular magnetic resonance (CMR) and computed tomography (CT) and newer techniques such as fusion imaging have proved to be efficient and reliable tools during pre-procedural planning in patients assessed for PPVR.
Topics: Cardiac Catheterization; Cardiac Surgical Procedures; Heart Defects, Congenital; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Treatment Outcome
PubMed: 35863726
DOI: 10.1016/j.hjc.2022.06.004 -
European Journal of Cardio-thoracic... May 2023After a Ross procedure, autograft failure can occur. At reoperation, repair of the autograft preserves the advantages of the Ross procedure. The aim of this...
OBJECTIVES
After a Ross procedure, autograft failure can occur. At reoperation, repair of the autograft preserves the advantages of the Ross procedure. The aim of this retrospective study was to assess mid-term results after reoperation of a failed autograft.
METHODS
Between 1997 and 2022, 30 consecutive patients (83% male; age 41 ± 11 years) underwent autograft reintervention between 60 days and 24 years (median 10 years) after a Ross procedure. The initial technique varied, full-root replacement (n = 25) being the most frequent. The indication for reoperation was isolated autograft regurgitation (n = 7), root dilatation (>43 mm) with (n = 17) or without (n = 2) autograft regurgitation, mixed dysfunction (n = 2) and endocarditis (n = 2). In 4 instances, the valve was replaced by valve (n = 1) or combined valve and root replacement (n = 3). Valve-sparing procedures consisted of isolated valve repair (n = 7) or root replacement (n = 19), and tubular aortic replacement. Cusp repair was performed in all but 2. Mean follow-up was 5.4 ± 6 years (35 days to 24 years).
RESULTS
Mean cross-clamp and perfusion times were 74 ± 26 and 132 ± 64 min. There were 2 perioperative deaths (7%; both valve replacement) and 2 patients died late (32 days to 1.2 years postoperatively). Freedom from cardiac death at 10 years was 96% after valve repair and 50% after replacement. Two patients required reoperation (1.68 and 16 years) following repair. One underwent valve replacement for cusp perforation, the other, root remodelling for dilatation. Freedom from autograft reintervention at 15 years was 95%.
CONCLUSIONS
Autograft reoperations after the Ross procedure can be performed as valve-sparing operations in the majority of cases. With valve-sparing, long-term survival and freedom from reoperation are excellent.
Topics: Humans; Male; Infant; Female; Reoperation; Autografts; Treatment Outcome; Retrospective Studies; Transplantation, Autologous; Dilatation, Pathologic; Aortic Valve; Aortic Valve Insufficiency; Pulmonary Valve; Follow-Up Studies
PubMed: 36971602
DOI: 10.1093/ejcts/ezad117 -
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 -
Frontiers in Cardiovascular Medicine 2022
PubMed: 35924216
DOI: 10.3389/fcvm.2022.970515 -
Journal of Medical Cases Mar 2020Carcinoid heart disease is an unusual cause of right heart failure. We present the case of a 53-year-old man with a history of metastatic carcinoid tumors originating...
Carcinoid heart disease is an unusual cause of right heart failure. We present the case of a 53-year-old man with a history of metastatic carcinoid tumors originating from the appendix, status post-chemotherapy, and liver embolizations. The patient presented with a right-sided valvular disease with severe pulmonic valve regurgitation, right ventricle dilation and pulmonary hypertension. He had carcinoid syndrome well controlled with long-acting lanreotide. He underwent tricuspid valve and pulmonary valve replacement with a stented bioprosthetic valve, maze and cardio-septal right ventricular outflow tract patch with an improvement of ventricular dysfunction. These findings supported the diagnosis of carcinoid heart disease presenting with pure right heart failure. The patient was symptom-free due to lanreotide, but ultimately, valve surgery is the preferred definite treatment in suitable patients. Carcinoid heart disease requires a high index of suspicion, and valve surgery is the only definitive treatment.
PubMed: 34434367
DOI: 10.14740/jmc3452 -
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 -
Archives of Gynecology and Obstetrics Nov 2022The purpose of this study was to explore the outcome in fetuses with prenatal diagnosis of absent pulmonary valve syndrome (APVS) on ultrasound imaging.
OBJECTIVE
The purpose of this study was to explore the outcome in fetuses with prenatal diagnosis of absent pulmonary valve syndrome (APVS) on ultrasound imaging.
METHODS
A manual web scraping technique was utilized, where MEDLINE and EMBASE were searched along the combination with other relevant medical subject headings such as "absent pulmonary valve syndrome", "prenatal APVS" and "APVS/outcome". The observed outcomes encompassed the rate of chromosomal abnormalities, associations and malformations linked to APVS and fetuses with APVS. A quality assessment of the included studies was also performed. We used meta-analyses of proportions to combine data and fixed or random-effects models according to the heterogeneity of the results.
RESULTS
Seven studies including 199 fetuses with APVS were included in the analysis. The median gestational age at referral to the tertiary center was 24.8 weeks. An association to tetralogy of Fallot (TOF) could be seen in 84.4% of all cases. In total 140 out of 199 cases underwent invasive testing, with a total number of 55 abnormal karyotypes [39.3% (95% CI 31.1-47.9%)]. 35.2% of the patients opted for termination of pregnancy (95% CI 28.5-42.3%).
CONCLUSION
The analysis underlines the distribution of fetuses with APVS, with 84.4% of cases presenting with TOF/APVS and only 12.6% having APVS/intact ventricular septum (IVS). Larger and more prospective study analyses is now needed, especially focusing on long-term follow-up periods of fetuses and children with APVS. Particularly as the postnatal course shows great variety depending on prenatal diagnosis.
Topics: Child; Female; Humans; Infant; Pregnancy; Prospective Studies; Pulmonary Atresia; Pulmonary Valve; Retrospective Studies; Tetralogy of Fallot; Ultrasonography, Prenatal
PubMed: 35043273
DOI: 10.1007/s00404-022-06397-4 -
Indian Journal of Thoracic and... Nov 2022The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of... (Review)
Review
The incidence of absent pulmonary valve (APV) in tetralogy of Fallot is 2.4 to 6.3%. About 1-3% of the patients with tetralogy of Fallot will have unilateral absence of pulmonary artery (UAPA). However, coexistence of APV with tetralogy of Fallot (TOF) and UAPA is extremely rare. This rare subset can present in two forms. In one group, the main pulmonary artery continues as either left or right pulmonary artery (UAPA) and there is true absence of contralateral pulmonary artery. The second group is termed as unilateral anomalous origin of pulmonary artery (UAOPA), in which the contralateral lung is supplied either by patent ductus arteriosus or a collateral from the aorta. There are a limited number of these cases in the literature. We present a rare case of TOF with APV and UAPA managed using a different surgical technique. Also, we have done contemporary literature review.
PubMed: 36258824
DOI: 10.1007/s12055-022-01393-4 -
Postepy W Kardiologii Interwencyjnej =... Jun 2021Percutaneous pulmonary valve replacement (PPVI) continues to gather pace in pediatric and adult congenital practice. This is fueled by an expanding repertoire of...
INTRODUCTION
Percutaneous pulmonary valve replacement (PPVI) continues to gather pace in pediatric and adult congenital practice. This is fueled by an expanding repertoire of devices, techniques and equipment to suit the heterogenous anatomical landscape of patients with lesions of the right ventricular outflow tract (RVOT). Contrast-induced nephropathy is a real risk for teenagers and adults with congenital heart disease (CHD).
AIM
To present a series of patients who underwent PPVI without formal RVOT angiography and propose case selection criteria for patients who may safely benefit from this approach.
MATERIAL AND METHODS
We retrospectively collected PPVI data from the preceding 2 years at our institution identifying patients who had been listed as suitable for consideration for contrast-free PPVI from our multidisciplinary team (MDT) meeting based on predefined criteria. Demographic, clinical, imaging and hemodynamic data were collected. Data were analyzed using SPSS.
RESULTS
Twenty-one patients were identified. All patients had a technically successful implantation with improvements seen in invasive and echocardiographic hemodynamic measurements. 90% of patients had a bio-prosthetic valve (BPV) prior to PPVI. One patient had a complication which may have been recognized earlier with post-intervention RVOT contrast injection.
CONCLUSIONS
Zero-contrast PPVI is technically feasible and the suitability criteria for those who might benefit are potentially straightforward. The advent of fusion and 3D imaging in cardiac catheterization laboratories is likely to expand our capacity to perform more procedures with less contrast. Patients with bio-prosthetic valves in the pulmonary position may benefit from contrast-free percutaneous pulmonary valve implantation.
PubMed: 34400923
DOI: 10.5114/aic.2021.107500 -
The Thoracic and Cardiovascular Surgeon Mar 2023Patients with congenital heart disease frequently require surgical or percutaneous interventional valve replacement after initial congenital heart defect (CHD)...
OBJECTIVES
Patients with congenital heart disease frequently require surgical or percutaneous interventional valve replacement after initial congenital heart defect (CHD) repair. In some of these patients, simultaneous replacement of both semilunar valves is necessary, resulting in increased procedural complexity, morbidity, and mortality. In this study, we analyze the outcomes of simultaneous aortic and pulmonary valve replacements following multiple surgical interventions for CHD.
METHODS
This was a retrospective study of 24 patients who after initial repair of CHD underwent single-stage aortic and pulmonary valve replacement at our institution between 2003 and 2021.
RESULTS
The mean age of the patients was 28 ± 13 years; the mean time since the last surgery was 15 ± 11 years. Decellularized valved homografts (DVHs) were used in nine patients, and mechanical valves were implanted in seven others. In eight patients, DVHs, biological, and mechanical valves were implanted in various combinations. The mean cardiopulmonary bypass time was 303 ± 104 minutes, and aortic cross-clamp time was 152 ± 73 minutes. Two patients died at 12 and 16 days postoperatively. At a maximum follow-up time of 17 years (mean 7 ± 5 years), 95% of the surviving patients were categorized as New York Heart Association heart failure class I.
CONCLUSIONS
Single-stage aortic and pulmonary valve replacement after initial repair of CHD remains challenging with substantial perioperative mortality (8.3%). Nevertheless, long-term survival and clinical status at the latest follow-up were excellent. The valve type had no relevant impact on the postoperative course. The selection of the valves for implantation should take into account operation-specific factors-in particular reoperability-as well as the patients' wishes.
PubMed: 36822229
DOI: 10.1055/a-2041-3528