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The Journal of Thoracic and... May 2023The Ross procedure is not commonly performed, owing to the procedural complexity and the risk of autograft and/or homograft reoperation. This study examined outcomes of...
BACKGROUND
The Ross procedure is not commonly performed, owing to the procedural complexity and the risk of autograft and/or homograft reoperation. This study examined outcomes of patients undergoing Ross reinterventions at a dedicated Ross center.
METHODS
We retrospectively reviewed 225 consecutive patients who underwent a Ross procedure between 1994 and 2019. Index and redo operation characteristics and outcomes were compared between patients with and those without redo operations. Multivariate analysis was used to identify independent predictors of Ross-related reinterventions. Survival was estimated with Kaplan-Meier analysis.
RESULTS
Sixty-six patients (29.3%) required redo Ross surgery, 41 patients (18.2%) underwent autograft reoperation only, 8 patients (3.6%) had a homograft reintervention, and 17 patients (7.6%) had both autograft and homograft reoperations (12 as a combined procedure and 5 as sequential procedures). The mean time to reintervention was 11 ± 6 years for autograft reoperations and 12 ± 7 years for homograft reoperations. Patients who underwent Ross-related reinterventions were younger (mean, 38 ± 11 years vs 43 ± 11 years; P < .01) and had a higher rate of New York Heart Association class III/IV (56% vs 38%; P = .02) at the index Ross procedure. Most patients undergoing autograft reintervention had aortic insufficiency and/or aneurysm (98.2%; 57 of 58). The primary reason for homograft reintervention was pulmonary stenosis (92%; 23 of 25). The operative mortality of Ross reintervention was 1.5% (1 of 66). Survival at 15 years was similar in patients who required a redo operation and those who did not (91.2% vs 93.9%; P = .23).
CONCLUSIONS
Ross reinterventions can be performed safely and maintain patients at the normal life expectancy restored by the index Ross procedure up to 15 years at experienced centers.
Topics: Humans; Heart Valve Prosthesis Implantation; Treatment Outcome; Retrospective Studies; Aortic Valve Insufficiency; Pulmonary Valve Stenosis; Reoperation; Aortic Valve; Pulmonary Valve; Aortic Valve Stenosis; Follow-Up Studies
PubMed: 36028359
DOI: 10.1016/j.jtcvs.2022.04.023 -
Journal of Veterinary Cardiology : the... Dec 2022Pulmonary artery dissection is a rare complication following balloon valvuloplasty for pulmonic stenosis. We sought to report the rate of this complication in dogs and...
INTRODUCTION/OBJECTIVES
Pulmonary artery dissection is a rare complication following balloon valvuloplasty for pulmonic stenosis. We sought to report the rate of this complication in dogs and describe the demographic, clinical, procedural, and outcome data in affected dogs.
ANIMALS, MATERIALS AND METHODS
Medical records at a single academic institution between 2002 and 2021 were reviewed for dogs with pulmonic stenosis treated by a balloon valvuloplasty. Dogs were included for evaluation if there was evidence of pulmonary artery dissection on echocardiography or necropsy following balloon valvuloplasty. The demographic, clinical, surgical, and follow-up information were then recorded.
RESULTS
Six dogs were included from 210 balloon valvuloplasty procedures for pulmonic stenosis giving a 3.9% rate of pulmonary dissection. There was a variety of signalment, pulmonary valve morphologies, and balloon catheter types used in each dog. All dogs had severe pulmonic stenosis (median pressure gradient of 208 mmHg, range 94-220 mmHg) with 5/6 dogs having a pressure gradient >144 mmHg. The median balloon to pulmonary valve annulus ratio was 1.35 (range 1.25-1.5). Three dogs died perioperatively, and three dogs were alive at follow up 3.3, 4.0, and 4.1 years postoperatively.
CONCLUSION
Pulmonary artery dissection is a rare complication following balloon valvuloplasty for pulmonic stenosis. Extreme elevations in preoperative pulmonary valve flow velocity were common. Prognosis is variable, with a potential 50% perioperative survival rate, but extended survival times were noted in those patients discharged from hospital.
Topics: Dogs; Animals; Balloon Valvuloplasty; Pulmonary Artery; Dog Diseases; Pulmonary Valve Stenosis; Echocardiography; Aortic Dissection; Treatment Outcome
PubMed: 36370467
DOI: 10.1016/j.jvc.2022.09.004 -
Congenital Heart Disease Sep 2019Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the...
OBJECTIVE
Cardiovascular lesions are the leading cause of morbidity and mortality in patients with Williams syndrome. Recent studies have rebutted conventional reports about the natural course of cardiovascular anomalies in Williams syndrome.
DESIGN
Retrospective study.
SETTING
Single tertiary center.
PATIENTS
Eighty patients with Williams syndrome followed up for more than 5 years.
INTERVENTIONS
Not applicable.
OUTCOME MEASURES
Long-term outcome of cardiovascular lesions, peak velocity change in obstructive cardiovascular lesions over time, post-interventional courses of disease-specific intervention, and intervention-free survival of obstructive cardiovascular lesions.
RESULTS
The median follow-up duration was 11.0 (5.1-28.3) years. Among 80 patients, supravalvular aortic stenosis (87.5%) was the most common cardiovascular lesion, followed by branch pulmonary stenosis (53.8%), mitral valve prolapse (22.5%), and aortic arch hypoplasia/coarctation (5.0%). During the follow-up period, the peak flow velocity of supravalvular aortic stenosis did not change on peak Doppler echocardiography. Initially, severe supravalvular aortic stenosis was aggravated (P < .027). Conversely, the peak velocity of branch pulmonary stenosis decreased (from 3.08 to 1.65 m/s; P < .001) within age 3.2 (0.4-6.9) years. Even the group with severe branch PS improved over time. Twenty-two patients (27.5%) with Williams syndrome underwent disease-specific interventions without mortality, mostly for supravalvular aortic stenosis or mitral valve prolapse. No patient in the late-onset and initially mild supravalvular aortic stenosis group needed intervention and 37.5%, 48.4%, and 65.1% in initially moderate and severe supravalvular aortic stenosis groups needed intervention at age 5, 10, and 20 years, respectively. Unlike the conventional therapeutic concept, the intervention for branch pulmonary stenosis was almost unnecessary.
CONCLUSIONS
In Williams syndrome, initially severe supravalvular aortic stenosis worsened over time and most branch pulmonary stenoses, including those in the severe group, improved spontaneously. Most patients with branch pulmonary stenosis did not require disease-specific intervention. Surgical repairs for cardiovascular abnormalities in Williams syndrome showed favorable results.
Topics: Adolescent; Cardiovascular Diseases; Child; Child, Preschool; Disease Progression; Echocardiography, Doppler; Female; Follow-Up Studies; Forecasting; Humans; Infant; Infant, Newborn; Male; Morbidity; Prognosis; Republic of Korea; Retrospective Studies; Survival Rate; Williams Syndrome
PubMed: 31166070
DOI: 10.1111/chd.12810 -
Circulation. Genomic and Precision... Aug 2023We aimed to examine clinical features and outcomes of consecutive molecularly characterized patients with Noonan syndrome with multiple lentigines and hypertrophic...
BACKGROUND
We aimed to examine clinical features and outcomes of consecutive molecularly characterized patients with Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy.
METHODS
A retrospective, longitudinal multicenter cohort of consecutive children and adults with a genetic diagnosis of Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy between 2002 and 2019 was assembled. We defined a priori 3 different patterns of left ventricular remodeling during follow-up: (1) an increase in ≥15% of the maximal left ventricular wall thickness (MLVWT), both in mm and -score (progression); (2) a reduction ≥15% of the MLVWT, both in mm and -score (absolute regression); (3) a reduction ≥15% of the MLVWT -score with a stable MLVWT in mm (relative regression). The primary study end point was a composite of cardiovascular death, heart transplantation, and appropriate implantable cardioverter defibrillator-shock.
RESULTS
The cohort comprised 42 patients with Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy, with a median age at diagnosis of 3.5 (interquartile range, 0.2-12.3) years. Freedom from primary end point was 92.7% (95% CI, 84.7%-100%) 1 year after presentation and 80.9% (95% CI, 70.1%-90.7%) at 5 years. Patients with MLVWT -score >13.7 showed reduced survival compared with those with <13.7. During a median follow-up of 3.7 years (interquartile range, 2.6-7.9), absolute regression was the most common type of left ventricular remodeling (n=9, 31%), followed by progression (n=6, 21%), and relative regression (n=6, 21%).
CONCLUSIONS
These findings provide insights into the natural history of left ventricular hypertrophy, and can help inform clinicians regarding risk stratification and clinical outcomes in patients with Noonan syndrome with multiple lentigines and hypertrophic cardiomyopathy.
Topics: Child; Adult; Humans; Infant; Child, Preschool; LEOPARD Syndrome; Retrospective Studies; Ventricular Remodeling; Cardiomyopathy, Hypertrophic; Noonan Syndrome
PubMed: 37199218
DOI: 10.1161/CIRCGEN.122.003861 -
Annals of Pediatric Cardiology 2020Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was...
BACKGROUND
Critical pulmonary stenosis (PS) is one of the life-threatening congenital heart diseases which present during the neonatal period with cyanosis. Surgical valvotomy was once the procedure of choice for critical PS; however, balloon pulmonary valvuloplasty (BPV) has now become the standard treatment. Although the procedure is usually simple, crossing the pulmonary valve from the femoral vein can be difficult, especially when severe tricuspid regurgitation and right atrium dilatation are present. In such patients, the maneuver can be simplified by using the right internal jugular vein approach. However, many operators are reluctant to use this approach because of unfamiliarity with the technique, potential complications, and paucity of reports. Until now, there is no literature describing BPV using the transjugular approach in neonates, also none directly comparing the transfemoral and transjugular approaches.
OBJECTIVE
We compared transjugular with the transfemoral approach in terms of procedure time and complications.
MATERIALS AND METHODS
This was a retrospective cohort study. Participants were neonates with critical PS undergoing BPV in the National Cardiovascular Center Harapan Kita from 2013 to 2018.
RESULTS
Of 15 neonates undergoing BPV, eight were done using the transjugular approach and seven using the femoral approach. Mean age and weight in both groups was similar. In all eight patients using transjugular approach, crossing the pulmonary valve was consistently quick and easy. The total procedural time, pulmonary crossing time, and fluoro time was significantly shorter using the transjugular approach (65 ± 8 vs. 108 ± 17.8 min, < 0.05; 22 ± 3.1 vs. 45 ± 14.8 min, < 0.01; 29 ± 13 vs. 67 ± 35 min, < 0.05). There were no complications relating to vascular access. Moreover, the BPV procedure itself demonstrated comparable results in both groups.
CONCLUSION
BPV using the transjugular approach is safe and effective to relieve critical PS in neonates compared to the transfemoral approach.
PubMed: 32030030
DOI: 10.4103/apc.APC_14_19 -
Heart (British Cardiac Society) Jul 2022Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on...
BACKGROUND
Complications and need for reinterventions are frequent in patients with pulmonary valve stenosis (PVS). Pulmonary regurgitation is common, but no data are available on outcome after pulmonary valve replacement (PVR).
METHODS
We performed a retrospective analysis of 215 patients with PVS who underwent surgical valvotomy or balloon valvuloplasty. Incidence and predictors of reinterventions and complications were identified. Right ventricle (RV) remodelling after PVR was also assessed.
RESULTS
After a median follow-up of 38.6 (30.9-49.4) years, 93% of the patients were asymptomatic. Thirty-nine patients (18%) had at least one PVR. Associated right ventricular outflow tract (RVOT) intervention and the presence of an associated defect were independent predictors of reintervention (OR: 4.1 (95% CI 1.5 to 10.8) and OR: 3.6 (95% CI 1.9 to 6.9), respectively). Cardiovascular death occurred in 2 patients, and 29 patients (14%) had supraventricular arrhythmia. Older age at the time of first intervention and the presence of an associated defect were independent predictors of complications (OR: 1.0 (95% CI 1.0 to 1.1) and OR: 2.1 (95% CI 1.1 to 4.2), respectively). In 16 patients, cardiac magnetic resonance before and after PVR was available. The optimal cut-off values for RV volume normalisation were 193 mL/m for RV end-diastolic volume indexed(sensitivity 80%, specificity 64%) and 100 mL/m for RV end-systolic volume indexed(sensitivity 80%, specificity 56%).
CONCLUSIONS
Previous RVOT intervention, presence of an associated defect and older age at the time of first repair were predictors of outcome. More data are needed to guide timing of PVR, and extrapolation of tetralogy of Fallot guidelines to this population is unlikely to be appropriate.
Topics: Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome; Ventricular Function, Right; Ventricular Remodeling
PubMed: 34815333
DOI: 10.1136/heartjnl-2021-320121 -
Revista Espanola de Cardiologia... Feb 2020
Topics: Humans; Pulmonary Valve Stenosis; Tetralogy of Fallot
PubMed: 31585848
DOI: 10.1016/j.rec.2019.07.012 -
Seminars in Perinatology Jun 2022Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right... (Review)
Review
Right ventricular outflow tract (RVOT) anomalies comprise a wide spectrum of congenital heart disease, typically characterized by obstruction to flow from the right ventricle to pulmonary arteries. This review highlights important considerations surrounding management strategy as well as clinical outcomes for the neonate with RVOT anomaly, including: pulmonary atresia with intact ventricular septum, congenital pulmonary valve stenosis, tetralogy of Fallot, and Ebstein anomaly with anatomic or physiologic RVOT obstruction.
Topics: Heart Defects, Congenital; Heart Ventricles; Humans; Infant, Newborn; Pulmonary Valve; Tetralogy of Fallot; Treatment Outcome; Ventricular Outflow Obstruction
PubMed: 35422353
DOI: 10.1016/j.semperi.2022.151583 -
Trends in Cardiovascular Medicine Feb 2022
Topics: Aortic Valve Stenosis; Heart Valve Prosthesis Implantation; Humans; Hypertension, Pulmonary
PubMed: 33400999
DOI: 10.1016/j.tcm.2020.12.008 -
European Journal of Medical Genetics Nov 2022Infants with Noonan Syndrome and hypertrophic cardiomyopathy have a poor prognosis and a high mortality especially when diagnosed before six months of age. As for the...
Infants with Noonan Syndrome and hypertrophic cardiomyopathy have a poor prognosis and a high mortality especially when diagnosed before six months of age. As for the majority of the RASopathies, no medical treatment has been approved for Noonan Syndrome. Meanwhile, several approved agents targeting the same RAS/MAPK signaling pathway are used in cancer treatment. In this case report we describe a child with Noonan Syndrome caused by a pathogenic RIT1 variant, who developed severe early-onset hypertrophic cardiomyopathy and pulmonary valve stenosis. She received off-label treatment with the MEK-inhibitor trametinib which resulted in complete remission of the cardiac hypertrophy and a significant improvement of the pulmonary valve stenosis. Our case emphasizes the potential of existing cancer agents targeting the RAS/MAPK signaling pathway as successful treatment for RASopathy manifestations.
Topics: Child; Female; Humans; Cardiomyopathy, Hypertrophic; Mitogen-Activated Protein Kinase Kinases; Mutation; Noonan Syndrome; Pulmonary Valve Stenosis; ras Proteins
PubMed: 36184070
DOI: 10.1016/j.ejmg.2022.104630