Aortic valve stenosis in alkaptonuric ochronosis

Alkaptonuria is a rare genetic disorder of tyrosine metabolism in which a bluish pigment accumulates in the connective tissues throughout the body, and causes degenerative changes. The most common clinical manifestation of ochronosis is arthropathy. Heart valves may also be affected, though cardiac involvement is rare. The present patient has cardiac ochronosis, and has several family members diagnosed with ochronosis and aortic valve stenosis.     

Aortic valve stenosis due to alkaptonuria

Cardiovascular disease is a less-well appreciated aspect of alkaptonuria. A 69-year-old man presented with shortness of breath and exertional chest pain. He had a previous diagnosis of alkaptonuria (endogenous ochronosis), confirmed on the basis of urine coloration, skin pigmentation and ochronotic arthropathy in the knees. Echocardiography and coronary angiography revealed severe aortic valve stenosis and concomitant coronary artery disease. The patient underwent biological aortic valve replacement (AVR) and coronary artery bypass grafting (CABG). Operative findings included ochronosis of a severely calcified aortic valve and the aortic intima, and bioprosthetic AVR and CABG were successfully performed.     

Aortic valve stenosis associated with Bazin's disease

Bazin's disease was first reported in 1861, and is described as erythema induratum or nodular vasculitis. The condition is seen occasionally in middle-aged women on the skin of the calf, and a relationship to a tuberculosis infection has been proposed. In the present patient, valvular lesions occurred simultaneously with Bazin's disease, with granulomatous changes being demonstrated by the aortic valve pathology.     

Aortic valve replacement for aortic stenosis in nonagenarians

We reviewed certain clinical and morphologic findings in 9 patients who had aortic valve replacement (AVR) for aortic stenosis (AS) when >or=90 years of age. All had AVR from February 2000 to April 2006. The aortic valve areas ranged from 0.41 to 1.00 cm2, and the transvalvular peak systolic gradients ranged from 20 to 110 mm Hg. The left ventricular ejection fractions were >or=50% in 6 of the 9 patients. The aortic valve was congenitally bicuspid in 3 patients, and the operatively excised valves in them weighed 4.20, 5.73, and 9.75 g, respectively (mean 6.56). The other 6 patients had 3-cuspid valves without commissural fusion, and the operatively excised valves in them weighed 0.43, 0.94, 1.08, 1.51, 1.98, and 4.43 g, respectively (median 1.30, mean 1.73). Coronary artery bypass grafting (CABG) was performed at the time of AVR in 8 of the patients. One patient died a day postoperatively and 2 others died 874 and 1,011 days, respectively, after operation. Two were in skilled nursing units postoperatively for several weeks. In conclusion, AS can be severe in nonagenarians and may be superimposed on a congenitally bicuspid aortic valve.     

Aortic valve stenosis: current physiopathological and therapeutic aspects

On the premise that the increased average life expectancy and a reduced incidence of rheumatic diseases have changed the epidemiological profile of valvulopathies, the paper analyses the causes, pathological anatomy, physiopathology and therapy of aortic valvular stenosis. First of all, the differences of acquired calcific stenosis, in which the orifice may be "forced" by the ventricular pump leading to a late onset of symptoms, are underlined. Clinical symptomatology must include angina, syncope or decompensation, rather than merely and elevated gradient, before surgical therapy is proposed. In addition to traditional therapeutic methods (valvular replacement) there is now the possibility of using balloon valvuloplasty which has provided good results in terms of increased valvular area and recovery of ventricular function; however, this is only a palliative step in cases where major surgery is contra-indicated.     

Aortic valve 'kissing' vegetation: mitral valve endocarditis with functional aortic stenosis

The case is reported of giant mitral valve vegetation that was wedged into the aortic valve plane (aortic valve 'kissing' vegetation), causing obstruction to the left ventricular outflow tract. In such a case, echocardiography plays a key role in the assessment of vegetation morphology, risk of embolization, impact on valvular function and indication for surgery. Because of its size, the obstructive hemodynamics, and the potential to induce aortic valve endocarditis, aortic 'kissing' vegetation requires surgical intervention to preserve the integrity of the valve and improve the hemodynamic status.     

Aortic valve damage caused by operative balloon dilatation of critical aortic valve stenosis

Operative balloon dilatation of the aortic valve was performed in seven neonates with critical stenosis of the aortic valve. The procedure was followed by the development of severe aortic regurgitation in four patients. Necropsy was performed in three and revealed partial detachment of the right coronary cusp of the aortic valve. Damage to the valve leaflet caused by balloon dilatation was probably the result of using a balloon with a diameter that was too large in relation to the aortic valve ring diameter and of shearing forces created in the aortic wall by the contracting ventricle. The diameter of the inflated balloon should not be larger than the diameter of the aortic valve ring.     

Aortic valve balloon dilatation in infants with critical aortic stenosis

Twenty-six infants aged 10 days-11 months (mean 3.5 +/- 2.4 months), with critical aortic stenosis underwent aortic valve balloon dilatation in last six years. In 19 (73%) infants, aortic valve balloon dilatation had to be performed as an emergency procedure. The systolic gradients decreased from 71.7 +/- 11.8 mm Hg to 21.0 +/- 8.1 mm Hg. There was no procedural death. Severe aortic regurgitation developed in two (7.7%) which was medically managed. Four (15.4%) infants had femoral artery thrombosis following aortic valve balloon dilatation and all responded to intravenous streptokinase. Congestive heart failure resolved in all (100%). Two infants developed endocarditis during follow-up after aortic valve balloon dilation and one of them died, another patient died of severe pulmonary artery hypertension. In the surviving 24 infants, left ventricular function improved markedly. On follow-up of 18 +/- 5 months, restenosis developed in two (7.7%) patients. We conclude that aortic valve balloon dilatation is safe and effective treatment for infants with critical aortic stenosis and severe left ventricular dysfunction.     

Aortic valvuloplasty using the transatrial route in aortic valve stenosis

We describe a new technique for aortic valvuloplasty in four patients. The balloon catheter was inserted percutaneously from the right femoral vein over a long guidewire introduced into the right femoral vein and advanced transseptally to the left atrium and left ventricle and drawn out of the body through the left femoral artery using an intravascular retriever set. With both ends of the long guidewire under manual control outside the body, the balloon could be easily fixed during inflation. The procedure was easy to perform and caused no complications.     

Aortic valve damage caused by operative balloon dilatation of critical aortic valve stenosis.

Operative balloon dilatation of the aortic valve was performed in seven neonates with critical stenosis of the aortic valve. The procedure was followed by the development of severe aortic regurgitation in four patients. Necropsy was performed in three and revealed partial detachment of the right coronary cusp of the aortic valve. Damage to the valve leaflet caused by balloon dilatation was probably the result of using a balloon with a diameter that was too large in relation to the aortic valve ring diameter and of shearing forces created in the aortic wall by the contracting ventricle. The diameter of the inflated balloon should not be larger than the diameter of the aortic valve ring.     

Aortic stenosis

Choice of the best surgical option for aortic stenosis (AS) must be individualized and requires discussion among patient, cardiologist, and surgeon to weigh the risks and benefits of different options. Mechanical valves have been preferred for young patients, for those with a life expectancy of more than 10 to 15 years, or for those who require anticoagulation. Bioprosthetic valves have been preferred for elderly patients, for patients with limited life expectancy, or for patients who are unable to be anticoagulated. Newer tissue valves (eg, the stentless porcine aortic bioprosthesis and homografts) as well as newer techniques (eg, the Ross procedure) have increased the number of available options and the complexity of the decision-making process.