Aortic Root Enlargement and Aortic Valve Replacement for Calcified Supravalvular and Valvular Aortic Stenosis in Homozygous Familial Hypercholesterolemia: A Case Report

2019 ◽  
Vol 11 (4) ◽  
pp. NP221-NP225 ◽  
Author(s):  
Ujjwal Kumar Chowdhury ◽  
Abhinavsingh Chauhan ◽  
Suruchi Hasija ◽  
Jhulana Kumar Jena ◽  
Lakshmi Kumari Sankhyan ◽  
...  
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Rare Condition ◽  
Mechanical Prosthesis ◽  
Valvular Aortic Stenosis ◽  
Aortic Root Enlargement ◽  
Familial Homozygous Hypercholesterolemia

Familial homozygous hypercholesterolemia is a rare disease with diverse clinical presentations ranging from premature ischemic heart disease to aortic root stenosis but rarely presents with anginal symptoms due to supravalvular and valvular aortic stenosis. We report a 19-year-old male patient with familial homozygous hypercholesterolemia with progressive supravalvular and valvular aortic stenosis that ultimately required aortic root enlargement and aortic valve replacement using a mechanical prosthesis, despite aggressive medical therapy. Surgical importance of this rare condition is highlighted.

scholarly journals Aortic Root Enlargement during Aortic Valve Replacement in Adults

2021 ◽  
pp. 56-62
Author(s):  
Miroslav M. Furman ◽  
Sergey V. Varbanets ◽  
Oleksandr M. Dovgan
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Severe Aortic Stenosis ◽  
Aortic Root Replacement ◽  
Safe Procedure ◽  
Aortic Pathology ◽  
Aortic Root Enlargement

Aortic valve replacement is a gold standard in the treatment of patients with severe aortic stenosis or combined aortic pathology. However, aortic valve pathology is often associated with a narrow aortic orifice, particularly in patients with severe aortic stenosis. In 1978, Rahimtoola first described the term of prosthesis-patient mismatch. He noted that effective orifice area of the prosthesis is smaller than that of the native valve. To minimize this complication, there are several surgical strategies: aortic root enlargement (ARE), implantation of a frameless biological prosthesis in the native position, neocuspidalization procedure, Ross procedure, aortic root replacement with xenograft or homograft. ARE is an excellent option, however, some authors outline additional perioperative risks. The aim. To analyze immediate results of ARE during isolated aortic valve replacement and in cases when it is combined with other heart pathologies. Materials and methods. Our study included 63 patients who underwent ARE. Isolated aortic valve replacement was performed in the majority of cases, but often aortic root replacement procedure was combined with coronary artery bypass grafting. Results and discussion. One of 63 patients died (hospital mortality 1.6%) at an early hospital stage (30 postoperative days). Measurement of the aortic valve ring was performed by two methods, through preoperative echocardiography and perioperative measurement using a valve sizer. However, perioperative dimension was chosen as the basis for the calculations. In 62 patients, the perioperative diameter of the aortic valve ring ranged from 19 to 23 mm, only one patient had a diameter of 24 mm. According to our findings, ARE enabled to achieve an average aortic ring size increase of 2.68 cm2 (from 1.5 to 3.4 cm2) and to prevent prosthesis-patient mismatch in 42 (66.7%) cases. Conclusions. Prosthesis-patient mismatch is considered a serious complication in the postoperative period. Narrow aortic root is a common pathology that should be considered during surgery. ARE is a safe procedure and is not associated with an increased risk of mortality and complications.

scholarly journals Tanned xenograft pericardium for aortic root enlargement during aortic valve replacement

1991 ◽  
Vol 17 (2) ◽  
pp. A361
Author(s):  
Donato Sisto ◽  
Sylvia Fernandes ◽  
Antonio Palma ◽  
Michele Nanna ◽  
Robert Frater
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Aortic Root Enlargement

scholarly journals Diagnosis and Management of Valvular Aortic Stenosis

2014 ◽  
Vol 8s1 ◽  
pp. CMC.S15716 ◽  
Author(s):  
Matthew J. Czarny ◽  
Jon R. Resar
Keyword(s):  
Aortic Valve ◽  
Aortic Stenosis ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
The United States ◽  
Surgical Aortic Valve Replacement ◽  
Transcatheter Aortic Valve ◽  
Rate Of Progression ◽  
Valvular Aortic Stenosis ◽  
Cardinal Symptoms

Valvular aortic stenosis (AS) is a progressive disease that affects 2% of the population aged 65 years or older. The major cause of valvular AS in adults is calcification and fibrosis of a previously normal tricuspid valve or a congenital bicuspid valve, with rheumatic AS being rare in the United States. Once established, the rate of progression of valvular AS is quite variable and impossible to predict for any particular patient. Symptoms of AS are generally insidious at onset, though development of any of the three cardinal symptoms of angina, syncope, or heart failure portends a poor prognosis. Management of symptomatic AS remains primarily surgical, though transcatheter aortic valve replacement (TAVR) is becoming an accepted alternative to surgical aortic valve replacement (SAVR) for patients at high or prohibitive operative risk.

scholarly journals Konno-rastan procedure combined with manougian root enlargement for small aortic root

2020 ◽  
Vol 4 (4) ◽  
pp. 18-21
Author(s):  
Metin Onur Beyaz ◽  
◽  
Nur Gizem Elipek ◽  
Ibrahim Demir ◽  
Didem Melis Oztas ◽  
...  
Keyword(s):  
Surgical Treatment ◽  
Aortic Valve ◽  
Aortic Stenosis ◽  
Valve Replacement ◽  
Aortic Root ◽  
Interventional Procedures ◽  
Pediatric Population ◽  
Patient Prosthesis Mismatch ◽  
Small Aortic Root ◽  
Aortic Root Enlargement

Aortic stenosis is a rarely seen condition in the pediatric population. Valve replacement is a treatment option for patients who do not benefit from medical or interventional procedures. In this report, we described our surgical treatment strategy in a 17-year-old patient who developed patient-prosthesis mismatch long after initial aortic valve replacement during the childhood period. Keywords: Konno-rastan procedure; manouguian technique; aortic root enlargement

scholarly journals Aortic Root Enlargement or Sutureless Valve Implantation?

2016 ◽  
Vol 4 (4) ◽  
pp. 742-743 ◽  
Author(s):  
Nikolaos G. Baikoussis ◽  
Panagiotis Dedeilias ◽  
Michalis Argiriou
Keyword(s):  
Aortic Valve ◽  
Aortic Valve Replacement ◽  
Valve Replacement ◽  
Aortic Root ◽  
Negative Impact ◽  
Term Survival ◽  
Old Patients ◽  
Sutureless Valve ◽  
Aortic Root Enlargement ◽  
Valve Implantation

Aortic valve replacement (AVR) in patients with a small aortic annulus is a challenging issue. The importance of prosthesis–patient mismatch (PPM) post aortic valve replacement (AVR) is controversial but has to be avoided. Many studies support the fact that PPM has a negative impact on short and long term survival. In order to avoid PPM, aortic root enlargement may be performed. Alternatively and keeping in mind that often some comorbidities are present in old patients with small aortic root, the Perceval S suturelles valve implantation could be a perfect solution. The Perceval sutureless bioprosthesis provides reasonable hemodynamic performance avoiding the PPM and providing the maximum of aortic orifice area. We would like to see in the near future the role of the aortic root enlargement techniques in the era of surgical implantation of the sutureless valve (SAVR) and the transcatheter valve implantation (TAVI).

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