Quadricuspid Aortic Valve

Abstract

To the Editor: Up to the present time, most cases with four-cusp aortic valve reported in the world literature were diagnosed at postmortem examination. The case here presented was diagnosed by aortography; there were three equal sized cusps and one smaller cusp. Moderate degree aortic insufficiency was an associated finding. A 44-year-old white woman was admitted to our hospital with complaints of exertional dyspnea and palpitation. There was no previous history of acute rheumatic fever. Physical examination revealed a well developed, well nourished patient. Her blood pressure was 140/60 mm Hg and the heart rate was regular, 90 per minute. The lungs were clear. The heart was enlarged and maximal impulse was felt at the sixth intercostal space outside the midclavicular line. There was a grade 3/6 blowing diastolic murmur audible over the entire precordium, with maximum point of intensity in the third left interspace parasternally. The liver was not enlarged and there was no ankle edema. X-ray examination of the chest showed moderate cardiomegaly and electrocardiogram revealed left ventricular hypertrophy. Other laboratory findings were within normal limits. Right heart catheterization revealed normal right sided pressure. Left heart catheterization showed no systolic gradient across the aortic valve. An aortic root angiogram showed moderate aortic insufficiency and four cusps of the aortic valve (three equal-sized cusps and one smaller cusp) were well observed (Fig 1). At surgery, four cusps were found, and an aortic valve replacement was inserted. Bicuspid aortic valve and unicuspid aortic valve are relatively common cardiac malformations, but a supernumerary aortic cusp is a rare anomaly. To date 207 cases of aortic or pulmonary quadricuspid valves have been reported, 192 pulmonary and 15 aortic.1Hurwitz LE Roberts WC Quadricuspid semilunar valve.Am J Cardiol. 1973; 31: 623Abstract Full Text PDF PubMed Scopus (296) Google Scholar The first case of quadricuspid aortic valve was reported in 1862 by Balington.2Robicsek F Sanger PW Daugherty HK Congenital quadricuspid aortic valve with displacement of the left coronary orifice.Am J Cardiol. 1969; 23 (Balington, quoted by): 288-290Abstract Full Text PDF PubMed Google Scholar According to Simonds,3Simonds JP Congenital malformations of the aortic and pulmonary valves.Am J Med Sci. 1923; 166: 584Crossref Google Scholar it occurs in less than 0.008 percent of the population. Of the 15 aortic cases mentioned in the world literature so far, most were diagnosed at postmortem examination. The first case to be diagnosed by aortography was reported by Peretz et al.4Peretz DL Changfoot GH Gourlay RH Four cusped aortic valve with significant hemodynamic abnormality.Am J Cardiol. 1969; 23: 291Abstract Full Text PDF PubMed Scopus (38) Google Scholar It should be added that quadricuspid semilunar valve, almost always a single malformation, is rarely associated with other congenital anomalies, such as atrial septal defect, ventricular septal defect, or patent ductus arteriosus. Seven anatomic variations of quadricuspid valves have been described by Hurwitz and Roberts,1Hurwitz LE Roberts WC Quadricuspid semilunar valve.Am J Cardiol. 1973; 31: 623Abstract Full Text PDF PubMed Scopus (296) Google Scholar the most common variation consisting of three equal sized cusps and one smaller one. Although the function of quadricuspid pulmonary valve is nearly always normal, quadricuspid aortic valves frequently function abnormally. Valvular insufficiency is the most common hemodynamic abnormality that is not observed in the early years of life.4Peretz DL Changfoot GH Gourlay RH Four cusped aortic valve with significant hemodynamic abnormality.Am J Cardiol. 1969; 23: 291Abstract Full Text PDF PubMed Scopus (38) Google Scholar Four-cusp aortic valve may be rarely demonstrated by aortography. Robicsek et al5Robicsek F Sanger PW Daugherty HK Congenital quadricuspid aortic valve with displacement of the left coronary orifice.Am J Cardiol. 1969; 23: 288-290Abstract Full Text PDF PubMed Scopus (53) Google Scholar reported a case wherein aortic insufficiency was diagnosed by aortography; however, quadricuspid aortic valve was only observed at operation. The first case diagnosed by aortography was reported by Peretz et al.4Peretz DL Changfoot GH Gourlay RH Four cusped aortic valve with significant hemodynamic abnormality.Am J Cardiol. 1969; 23: 291Abstract Full Text PDF PubMed Scopus (38) Google Scholar Our own case was also diagnosed by aortography.