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World Journal of Cardiology May 2021Right ventricular outflow tract (RVOT) obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot, pulmonary atresia/stenosis... (Review)
Review
Right ventricular outflow tract (RVOT) obstruction is present in a variety of congenital heart disease states including tetralogy of Fallot, pulmonary atresia/stenosis and other conotruncal abnormalities After surgical repair, these patients develop RVOT residual abnormalities of pulmonic stenosis and/or insufficiency of their native outflow tract or right ventricle to pulmonary artery conduit. There are also sequelae of other surgeries like the Ross operation for aortic valve disease that lead to right ventricle to pulmonary artery conduit dysfunction. Surgical pulmonic valve replacement (SPVR) has been the mainstay for these patients and is considered standard of care. Transcatheter pulmonic valve implantation (TPVI) was first reported in 2000 and has made strides as a comparable alternative to SPVR, being approved in the United States in 2010. We provide a comprehensive review in this space-indications for TPVI, detailed procedural facets and up-to-date review of the literature regarding outcomes of TPVI. TPVI has been shown to have favorable medium-term outcomes free of re-interventions especially after the adoption of the practice of pre-stenting the RVOT. Procedural mortality and complications are uncommon. With more experience, recognition of risk of dreaded outcomes like coronary compression has improved. Also, conduit rupture is increasingly being managed with transcatheter tools. Questions over endocarditis risk still prevail in the TPVI population. Head-to-head comparisons to SPVR are still limited but available data suggests equivalence. We also discuss newer valve technologies that have limited data currently and may have more applicability for treatment of native dysfunctional RVOT substrates.
PubMed: 34131475
DOI: 10.4330/wjc.v13.i5.117 -
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 -
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 -
Multimedia Manual of Cardiothoracic... Nov 2023The Ross-Personalized External Aortic Root Support procedure is a surgical aortic valve replacement technique in which the autologous pulmonary valve is transposed in...
The Ross-Personalized External Aortic Root Support procedure is a surgical aortic valve replacement technique in which the autologous pulmonary valve is transposed in the aortic position to replace the malfunctioning aortic valve and a homograft is implanted in the pulmonary position. To prevent autograft dilatation, a Personalized External Aortic Root Support prosthesis is included in the proximal autograft anastomosis and wrapped around the ascending aorta. The aorta is transected transversely, the aortic valve is resected, and the coronary arteries are mobilized and cut out of the sinuses, leaving a rim. The pulmonary autograft is harvested by transecting the pulmonary artery and part of the right ventricular outflow tract. The autograft is approximated to the aortic root and inverted inside the ventricle. The proximal anastomosis is performed including the prosthesis between the aortic root and the autograft. The coronary buttons are threaded through appropriately positioned and sized holes in the prosthesis and reimplanted into the autograft. The ascending aorta is appropriately adapted and anastomosed with the distal autograft. When the patient is off cardiopulmonary bypass, the prosthesis can be closed longitudinally and is anchored to the distal aortic adventitia.
Topics: Humans; Autografts; Aorta, Thoracic; Transplantation, Autologous; Aortic Valve; Aorta; Aortic Valve Stenosis; Aortic Valve Insufficiency; Pulmonary Valve; Heart Valve Prosthesis Implantation; Reoperation
PubMed: 37942704
DOI: 10.1510/mmcts.2023.077 -
CJC Pediatric and Congenital Heart... Dec 2023Surgical treatment of tetralogy of Fallot (TOF) involves surgical relief of right ventricular outflow tract (RVOT) obstruction and closure of ventricular septal defect.... (Review)
Review
Surgical treatment of tetralogy of Fallot (TOF) involves surgical relief of right ventricular outflow tract (RVOT) obstruction and closure of ventricular septal defect. However, some patients may require staged palliation before surgical repair. This traditionally was achieved only with surgery but recently evolved to include catheter-based techniques. RVOT dysfunction occurs inevitably after the surgical repair of TOF and, depending on the surgical approach, manifests as either progressive stenosis, regurgitation, or a combination of both. This predisposes the individual to repeated RVOT interventions with the attendant risks of multiple open-heart surgeries. The advent of transcatheter pulmonary valve replacement has reduced the operative burden, and the expansion of transcatheter pulmonary valve replacement device platforms has widened the type and size of RVOT anatomies that can be treated. This review will discuss the transcatheter therapies available throughout the lifespan of the patient with TOF.
PubMed: 38161670
DOI: 10.1016/j.cjcpc.2023.09.005 -
Anatolian Journal of Cardiology Apr 2020
Topics: Aneurysm; Computed Tomography Angiography; Diagnosis, Differential; Dyspnea; Humans; Male; Middle Aged; Pulmonary Artery; Pulmonary Valve Stenosis
PubMed: 32352412
DOI: 10.14744/AnatolJCardiol.2020.57598 -
Prenatal features of ductus arteriosus-related branch pulmonary stenosis in fetal pulmonary atresia.Ultrasound in Obstetrics & Gynecology :... Sep 2021Ductus arteriosus (DA)-related branch pulmonary stenosis (PS), due to ductal tissue migration into the proximal pulmonary artery (PA) ipsilateral to the DA, is common in...
OBJECTIVE
Ductus arteriosus (DA)-related branch pulmonary stenosis (PS), due to ductal tissue migration into the proximal pulmonary artery (PA) ipsilateral to the DA, is common in newborns with pulmonary atresia (PAtr) and contributes significantly to their mortality and morbidity. We sought to define fetal echocardiographic predictors of DA-PS in PAtr.
METHODS
This was a study of all neonates diagnosed prenatally with PAtr and a DA-dependent pulmonary circulation, with a DA that joined the underbelly of the arch, who had undergone surgical or catheter intervention in our hospital between 2009 and 2018. The postnatal echocardiograms and clinical records were reviewed to confirm the presence or absence of DA-PS based on the need for angioplasty at initial intervention and/or development of proximal PA stenosis post intervention. Fetal echocardiograms were examined for the features of DA-PS.
RESULTS
Of 53 fetuses with PAtr, 34 (64%) had analyzable images, including 20/34 (59%) with and 14/34 (41%) without DA-PS. An inability to visualize the branch PAs in the same plane, largely associated with abnormal DA insertion into the ipsilateral PA (85% of cases), had sensitivity, specificity and positive (PPV) and negative (NPV) predictive values of 75%, 100%, 100% and 74%, respectively, for the prediction of postnatal DA-PS. The mean branch PA posterior bifurcation angle was more obtuse in cases with DA-PS compared to cases without DA-PS (117° ± 17° vs 79° ± 17°, P < 0.001), and an angle of > 100°, the preoperative cut-off observed previously in affected newborns, had a sensitivity, specificity, PPV and NPV of 88%, 79%, 82% and 85%, respectively. The receiver-operating-characteristics curve revealed an angle of ≥ 105° to have a sensitivity and specificity of 88% and 93%, respectively, for prenatal prediction of DA-PS. The presence of one or both features (inability to image in the same plane and the posterior bifurcation angle of ≥ 105°) had a sensitivity, specificity, PPV and NPV of 100%, 93%, 95% and 100%, respectively.
CONCLUSION
An inability to visualize the branch PAs in the same plane, associated with abnormal insertion of the DA in most cases, and/or the presence of a posterior PA bifurcation angle of ≥ 105° are predictive features of postnatal DA-PS in fetuses with PAtr. © 2020 International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Adult; Ductus Arteriosus; Echocardiography; Female; Fetus; Gestational Age; Humans; Infant, Newborn; Male; Predictive Value of Tests; Pregnancy; Pulmonary Artery; Pulmonary Atresia; Pulmonary Valve Stenosis; Ultrasonography, Prenatal
PubMed: 33347665
DOI: 10.1002/uog.23576 -
Pediatric Cardiology Jun 2022Gestational viral infection has been associated with congenital heart disease (CHD). Few studies, however, have studied the potential role of gestational Coxsackievirus...
Gestational viral infection has been associated with congenital heart disease (CHD). Few studies, however, have studied the potential role of gestational Coxsackievirus B (CVB) exposure in the pathogenesis of CHD. We prospectively enrolled women with pregnancies affected by CHD to explore possible associations with in utero CVB exposure. Serum samples were obtained from 122 women referred for fetal echocardiography between 2006 and 2018. We quantified CVB IgG and IgM levels, with titers ≥ 15.0 U/mL considered positive and measured neutralizing antibodies for three CVB serotypes: CVB1, CVB3, and CVB4. Using data from the national enterovirus surveillance system, we compared the annual exposure rates for each serotype in our cohort to infections reported across the United States. 98 pregnancies with no genetic defects were included. Overall, 29.6% (29/98) had positive IgG and 4.1% (4/98) of women had positive CVB IgM titers. To explore first-trimester CVB exposure, we focused exclusively on the 26 women with positive IgG and negative IgM titers. 61.5% (16/26) had neutralizing antibodies against a single serotype and 38.5% (10/26) against multiple CVB serotypes. CVB4 neutralizing antibodies were the most common (65.4%, 17/26), followed by CVB3 (53.9%, 14/26) and CVB1 (30.8%, 8/26). Among these, 30.8% of babies presented pulmonary valve anomalies: 19.2% (5/26) pulmonary atresia, and 11.5% (3/26) pulmonary stenosis. 23.1% (6/26) of babies had coronary sinusoids. CVB exposure in our cohort mirrored that of reported infections in the United States. Our results suggest a possible association between gestational CVB exposure and specific CHD, particularly pulmonary valve anomalies and coronary sinusoids.
Topics: Antibodies, Neutralizing; Antibodies, Viral; Coxsackievirus Infections; Enterovirus B, Human; Female; Heart Defects, Congenital; Humans; Immunoglobulin G; Immunoglobulin M; Pulmonary Atresia
PubMed: 35022808
DOI: 10.1007/s00246-021-02805-9 -
Frontiers in Pediatrics 2021Pulmonary valve (PV) stenosis affects cardiac pulmonary function and exercise performance. A cardiopulmonary exercise test (CPET) combined with a transthoracic...
OBJECTIVE
Pulmonary valve (PV) stenosis affects cardiac pulmonary function and exercise performance. A cardiopulmonary exercise test (CPET) combined with a transthoracic echocardiogram (TTE) can measure exercise performance, disease progression, and treatment effects. We assessed the exercise capacity in children with PV stenosis by conducting CPET and TTE.
METHODS
From 2005 to 2021, 84 patients with PV stenosis aged 6-18 years were enrolled; 43 were treated with balloon pulmonary valvuloplasty (BPV) (Group A), and 41 received follow-up care (Group B), and their CPET and pulmonary function test results were compared with 84 healthy, matched individuals (Control). We also conducted TTE to compare the peak pulmonary artery pulse wave velocity and pulmonary valve (PV) area before and after catheterization and follow-up care.
RESULTS
There were no significant differences among the CPET parameters of the patient groups and controls in anaerobic metabolic equivalent (MET) (group A: 6.44 ± 0.58; group B: 6.28 ± 0.47, control: 6.92 ± 0.39, = 0.110), peak MET (group A: 9.32 ± 0.74; group B: 9.13 ± 0.63; control: 9.80 ± 0.52, = 0.263), and heart rate recovery (group A: 28.04 ± 4.70; group B: 26.44 ± 3.43, control:26.10 ± 2.42, = 0.718). No significant differences were found in the pulmonary functions between the three groups. The pulmonary artery pulse wave velocity significantly decreased after catheterization (3.97 ± 1.50 vs. 1.95 ± 0.94, < 0.0001), but not after follow-up care (1.67 ± 0.77 vs. 1.75 ± 0.66, = 0.129). The pulmonary vale area significantly improved in group A (0.89 ± 0.71 vs. 1.16 ± 0.58, < 0.0001), whereas only insignificant progression of PV stenosis was observed in group B (1.60 ± 0.64 vs. 1.57 ± 0.65, = 0.110).
CONCLUSIONS
Patients treated with BPV had a similar exercise capacity with that of patients under follow-up care and the healthy controls. Larger or multi-center studies should be conducted to confirm the physical fitness of pediatric patients with PV stenosis after management.
PubMed: 35096713
DOI: 10.3389/fped.2021.802645 -
Journal of the American Heart... Dec 2021Background Degenerative aortic valve (AoV) disease and resulting aortic stenosis are major clinical health problems. Murine models of valve disease are rare, resulting...
Background Degenerative aortic valve (AoV) disease and resulting aortic stenosis are major clinical health problems. Murine models of valve disease are rare, resulting in a translational knowledge gap on underlying mechanisms, functional consequences, and potential therapies. Naïve New Zealand obese (NZO) mice were recently found to have a dramatic decline of left ventricular (LV) function at early age. Therefore, we aimed to identify the underlying cause of reduced LV function in NZO mice. Methods and Results Cardiac function and pulmonary hemodynamics of NZO and age-matched C57BL/6J mice were monitored by serial echocardiographic examinations. AoVs in NZO mice demonstrated extensive thickening, asymmetric aortic leaflet formation, and cartilaginous transformation of the valvular stroma. Doppler echocardiography of the aorta revealed increased peak velocity profiles, holodiastolic flow reversal, and dilatation of the ascending aorta, consistent with aortic stenosis and regurgitation. Compensated LV hypertrophy deteriorated to decompensated LV failure and remodeling, as indicated by increased LV mass, interstitial fibrosis, and inflammatory cell infiltration. Elevated LV pressures in NZO mice were associated with lung congestion and , evident as right ventricular dilatation, decreased right ventricular function, and increased mean right ventricular systolic pressure, indicative for the development of pulmonary hypertension and ultimately right ventricular failure. Conclusions NZO mice demonstrate as a novel murine model to spontaneously develop degenerative AoV disease, aortic stenosis, and the associated end organ damages of both ventricles and the lung. Closely mimicking the clinical scenario of degenerative AoV disease, the model may facilitate a better mechanistic understanding and testing of novel treatment strategies in degenerative AoV disease.
Topics: Animals; Aortic Valve Disease; Aortic Valve Stenosis; Disease Models, Animal; Mice; Mice, Inbred C57BL; Mice, Obese; New Zealand
PubMed: 34779224
DOI: 10.1161/JAHA.121.023131