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Kardiologiia Mar 2020LEOPARD syndrome with multiple lentigines (cardiomyopathic lentiginosis) is a rare, genetically predetermined disease with autosomal dominant inheritance. Prevalence of...
LEOPARD syndrome with multiple lentigines (cardiomyopathic lentiginosis) is a rare, genetically predetermined disease with autosomal dominant inheritance. Prevalence of this syndrome is unknown. One of pathognomonic clinical manifestations of this syndrome is the presence of multiple lentiginous pigment spots all over the body. The most common cardiac manifestation (approximately 80%) is myocardial hypertrophy. We presented a rare clinical case of detecting LEOPARD syndrome with multiple lentigines in a 32-year old female patient with major manifestations evident as pronounces morpho-functional alterations, myocardial hypertrophy, and heart rhythm disorders.
Topics: Adult; Female; Heart; Humans; LEOPARD Syndrome
PubMed: 32375626
DOI: 10.18087/cardio.2020.3.n944 -
World Journal For Pediatric &... May 2021Stented bioprosthesis implant at surgical pulmonary valve replacement (PVR) ideally should be 25 to 27 mm to facilitate future percutaneous PVR. This often requires...
BACKGROUND
Stented bioprosthesis implant at surgical pulmonary valve replacement (PVR) ideally should be 25 to 27 mm to facilitate future percutaneous PVR. This often requires accommodating 35 to 37 mm diameter sewing ring in the pulmonary position and requires anterior patch augmentation of the right ventricular outflow tract (RVOT). We present a novel "interposition" technique of PVR that allows upsizing the valve without RVOT patch augmentation.
METHODS
Using standard cardiopulmonary bypass, the main pulmonary artery (MPA) is dissected and transected at an appropriate level. The remnants of pulmonary valve leaflets are excised. The valve stent posts are telescoped into distal MPA, the MPA continuity is restored by end-to-end anastomosis of the proximal and distal MPA, with the interposed prosthetic valve sewing ring in the suture line between the two edges of the MPA with the bulk of the sewing ring extravascular.
RESULT
A total of seven patients (tetralogy of Fallot, three; congenital pulmonary stenosis, four; age range: 15-33 years) underwent the procedure. No patient required RVOT patch augmentation, all patients were extubated in the operating room and were fast-tracked to recovery. Our proposed technique of PVR has the following advantages: accommodate larger size valve, eliminates risk of a paravalvar leak, coronary compression, and anterior tilting of the prosthesis.
CONCLUSION
The valve interposition technique avoids the need for RVOT patch, allows implantation of an adequate sized prosthetic valve, maintains native geometry of the pulmonary artery without the risk of tilting of the prosthesis, and eliminates the risk of paravalvular regurgitation and left coronary compression.
Topics: Adolescent; Adult; Cardiac Catheterization; Heart Valve Prosthesis; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Retrospective Studies; Tetralogy of Fallot; Treatment Outcome; Young Adult
PubMed: 33942689
DOI: 10.1177/21501351211000283 -
Indian Journal of Pediatrics Jan 2024
Topics: Humans; Catheterization; Pulmonary Valve Stenosis; Follow-Up Studies
PubMed: 37806996
DOI: 10.1007/s12098-023-04874-5 -
Pediatric Cardiology Mar 2022Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for isolated pulmonary valve stenosis. While this procedure is highly efficacious and has...
Percutaneous balloon pulmonary valvuloplasty (PBPV) is the treatment of choice for isolated pulmonary valve stenosis. While this procedure is highly efficacious and has an excellent safety profile, as currently practiced, patients are obligatorily exposed to the secondary risks of ionizing radiation and contrast media. To mitigate these risks, we developed a protocol which utilized echo guidance for portions of the procedure which typically require fluoroscopy and/or angiography. Ten cases of echo-guided pulmonary valvuloplasty (EG-PBPV) for isolated pulmonary stenosis in children less than a year of age were compared to a historical cohort of nineteen standard cases using fluoroscopy/angiography alone, which demonstrated equivalent procedural outcomes and safety, while achieving a median reduction in radiation (total dose area product) and contrast load of 80% and 84%, respectively. Our early experience demonstrates that EG-PBPV in neonates and infants has results equivalent to standard valvuloplasty but with less radiation and contrast.
Topics: Balloon Valvuloplasty; Cardiac Surgical Procedures; Child; Fluoroscopy; Humans; Infant; Infant, Newborn; Pulmonary Valve Stenosis; Treatment Outcome
PubMed: 34839381
DOI: 10.1007/s00246-021-02771-2 -
Pediatric Radiology Dec 2022Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although... (Review)
Review
Tetralogy of Fallot (TOF) is the most common cause of cyanotic congenital heart disease (CHD) and the most frequent complex CHD encountered in adulthood. Although children with TOF share four characteristic features (subaortic ventricular septal defect, overriding aorta, right ventricular hypertrophy, pulmonary stenosis), the clinical spectrum and course are in fact greatly heterogeneous. Echocardiography remains the mainstay for diagnosis, presurgical planning and postoperative follow-up. However, with continued technological advances, CT now plays an increasing role in TOF evaluation and management, helping to minimize routine invasive catheter angiography. Preoperatively, CT is uniquely suited to assess associated pulmonary arterial, aortic and coronary anomalies as well as extra-cardiovascular structures and is particularly helpful for delineating complex anatomy in the TOF subtypes of absent pulmonary valve and pulmonary atresia with major aortopulmonary collaterals. Postoperatively, CT is useful for identifying surgical complications and for long-term monitoring including volumetry quantification, especially in children for whom MRI is contraindicated or limited by implanted devices such as pacemakers and stents. In this article, we review key clinical features and considerations in the pre- and postoperative TOF patient and the burgeoning role of CT for facilitating accurate diagnosis and personalized intervention.
Topics: Child; Humans; Adult; Tetralogy of Fallot; Pulmonary Atresia; Pulmonary Artery; Aorta; Tomography, X-Ray Computed
PubMed: 34427695
DOI: 10.1007/s00247-021-05179-5 -
Journal of the American College of... Jan 2021Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has... (Review)
Review
Transcatheter balloon valvuloplasty for the treatment of aortic and pulmonary valve stenosis was first described nearly 40 years ago. Since that time, the technique has been refined in an effort to optimize acute outcomes while reducing the long-term need for reintervention and valve replacement. Balloon pulmonary valvuloplasty is considered first-line therapy for pulmonary valve stenosis and generally results in successful relief of valvar obstruction. Larger balloon to annulus (BAR) diameter ratios can increase the risk for significant valvar regurgitation. However, the development of regurgitation resulting in right ventricular dilation and dysfunction necessitating pulmonary valve replacement is uncommon in long-term follow-up. Balloon aortic valvuloplasty has generally been the first-line therapy for aortic valve stenosis, although some contemporary studies have documented improved outcomes following surgical valvuloplasty in a subset of patients who achieve tri-leaflet valve morphology following surgical repair. Over time, progressive aortic regurgitation is common and frequently results in the need for aortic valve replacement. Neonates with critical aortic valve stenosis remain a particularly high-risk group. More contemporary data suggest that acutely achieving an aortic valve gradient <35 mm Hg with mild aortic regurgitation may improve long-term valve performance and reduce the need for valve replacement. Continued study will help to further improve outcomes and reduce the need for future reinterventions.
Topics: Aortic Valve Insufficiency; Aortic Valve Stenosis; Balloon Valvuloplasty; Heart Defects, Congenital; Heart Valve Prosthesis Implantation; Humans; Pulmonary Valve; Pulmonary Valve Insufficiency; Pulmonary Valve Stenosis
PubMed: 33413944
DOI: 10.1016/j.jacc.2020.10.052 -
Folia Medica Aug 2022Watson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown...
Watson for the first time reported a case series of children in a family that presented with pulmonary valve stenosis, mental retardation, short stature, and small brown color skin lesions that are known as cafe-au-lait spots. We present a rare new variant of the syndrome in an adult patient with severe pulmonary valve stenosis, main, left, and right pulmonary artery aneurysm, short stature, mental retardation, coronary artery disease, and skin lesions. The patient underwent open cardiac surgery with pulmonary valvotomy and aneurysmorrhaphy of the main pulmonary artery and its right and left branches. The postoperative course was uneventful and the six-month follow-up with transthoracic echocardiography revealed no recurrence of aneurysm of repairing pulmonary arteries and good clinical outcome of the patient. Our patient had a unique characteristic of aneurysm of the main pulmonary artery and its both branches that has rarely been reported previously in the medical literature.
Topics: Adult; Aneurysm; Child; Humans; Intellectual Disability; Neurofibromatosis 1; Pulmonary Artery; Pulmonary Valve Stenosis
PubMed: 36045473
DOI: 10.3897/folmed.64.e63559 -
The Journal of Thoracic and... May 2022
Topics: Humans; Philosophy; Pulmonary Valve Stenosis
PubMed: 34756430
DOI: 10.1016/j.jtcvs.2021.10.016 -
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