A case of pentacuspid pulmonary valve

Surg Radiol Anat (2010) 32:613–615 DOI 10.1007/s00276-009-0607-7 A N A T O M I C V A R I A T IO N S A case of pentacuspid pulmonary valve Sema Demircin · Nigar Keles-Coskun Received: 26 October 2009 / Accepted: 1 December 2009 / Published online: 19 December 2009  Springer-Verlag 2009 Abstract A pentacuspid pulmonary valve is a rare congenital anomaly. The present report describes a case of pentacuspid pulmonary valve encountered during the autopsy of a 50-year-old man with a ruptured abdominal aortic aneurysm. The pentacuspid pulmonary valve had three relatively equal cusps and two smaller cusps. In this case, a tricuspid aortic valve was identiWed with normal structure and no evidence of heart failure was found clinically or pathologically. Keywords anomalies Pentacuspid pulmonary valve · Valve Introduction The pulmonary valve has three semilunar leaXets or cusps attached by convex edges partly to the infundibular wall of the right ventricle and partly to the beginning of the pulmonary trunk. The oYcial nomenclature of Terminologia Anatomica [22] refers to an anterior, a posterior and a septal cusp, based on their position in the foetus. The position changes with development and in the adult there are two anterior cusps, right and left (valvula semilunaris dextra and sinistra) and a posterior one (valvula semilunaris posS. Demircin Council of Forensic Medicine, Antalya Group Authority, Antalya, Turkey S. Demircin Department of Forensic Medicine, Faculty of Medicine, Akdeniz University, 07059 Antalya, Turkey N. Keles-Coskun (&) Department of Anatomy, Faculty of Medicine, Akdeniz University, 07070 Antalya, Turkey e-mail: nigarc@akdeniz.edu.tr terior) [9, 22]. Congenital anomalies of the semilunar valves are rare and most often involve the presence of a monocuspid [12], bicuspid [6, 11, 21] or quadricuspid [10, 12, 18, 19, 21, 23] valves of the pulmonary and aortic valves. Apart from quadricuspid valves found in the setting of the common arterial trunk, quadricuspid semilunar valves are exceedingly uncommon, with the pulmonary valve being aVected more frequently than the aortic valve. When the pulmonary valve is involved, its abnormal architecture rarely alters the function of the valve and the anomaly often remains silent [15]. Bicuspid and quadricuspid pulmonary valves are usually considered as minor cardiac defects because of their clinical relevance [7]. Embryologically, by the fourth week of gestation, a pair of bulbar ridges forms in the cephaled portion of the truncus arteriosus. Similar ridges form in the truncus arteriosus which are continuous with the bulbar ridges. The spiral orientations of the bulbar and truncal ridges, possibly caused by the streaming of blood from the ventricles, result in the formation of a spiral aorticopulmonary septum when the bulbar and the truncal ridges fuse. This septum divides the bulbus cordis and truncus arteriosus into two arterial channels: aorta and pulmonary trunk. The results of experimental studies suggest that neural crest cells substantially contribute to the development of the aorticopulmonary septum as well as to other parts of the heart [14]. By sixth week, the division of arteriosus is completed, and then aorta and pulmonary trunk begin to appear. The semilunar valves are formed by mesenchymal outgrowth from the proliferations of two bulbar ridges and intercalated valvular swellings. Normally, three outgrowths bulge into the lumen of the great vessel and develop into three semilunar cusps that coapt in the centre of the valve oriWce. An abnormal number of cusps result from developmental changes in the early stages of truncal separation [11, 17]. 123 614 Fig. 1 Pulmonary valve with Wve cusps According to literature, there has been no previous report of a pentacuspid pulmonary valve. Hence, the present case is the Wrst report of such valve and can be embryogenetically interesting. Case report During the autopsy study of a 50-year-old male with a ruptured abdominal aortic aneurysm, the pulmonary valve was dissected from the anterior part of the heart and a pentacuspid pulmonary valve was encountered. The pentacuspid pulmonary valve had three relatively equal (2.5–3 cm) cusps and two smaller (2 cm) cusps (Fig. 1). On the other hand, the aortic valve was in normal anatomic structure. In this case, no clinical or biological evidence of heart failure was found. Moreover, no pulmonary stenosis and regurgitation were identiWed because of the diVerent pulmonary valve structure. Discussion Usually, pulmonary valve has three leaXets, but some may have two, four or more. The lesion is due to a failure in development of the aorticopulmonary septum, and is almost certainly linked to abnormal migration of cells into the heart from the neural crest [1, 9]. The embryological base of this pentacuspid pulmonary valve anomaly is not well documented. Embryological observations in animals [7, 8] and extrapolation of the explanations for quadricuspid aortic and pulmonary valves oVer several possibilities [3, 11, 21]. Although embryological development is a continuous 123 Surg Radiol Anat (2010) 32:613–615 process, three critical phases with regard to the supernumerary-cusped aortic or pulmonary valve can be recognised [21]. According to Simonds [21] (1) supernumerary prevalvular pads in the embryological truncus arteriosus may evolve in supernumerary-cusped aortic or pulmonary valves after separation into aorta and pulmonary artery; (2) a normal number of prevalvular pads in the truncus arteriosus may become excessively divided because of an abnormal dividing pattern of aorta and pulmonary artery, thereby producing a supernumerary-cusped aortic or pulmonary valve; (3) after normal separation of the aorta and pulmonary artery, the prevalvular pads might develop in an abnormal way to form a supernumerary-cusped valve. The Wrst or second explanation of Simonds [21] will become more probable in the Boger’s case report [3]. In this paper, we can speculate that the second or the third explanation is more probable that the aortic valve was in normal structure in the present case. Embryological abnormal pulmonary valves are generally linked with early stages of Fallot’s tetralogy [2]. They are caused by an arrest in heart development and an abnormal degree of coarctation of the aorta. The evidence from embryos supports the general belief that abnormal pulmonary cusps of all ages, when not caused by infection, are of congenital origin [20]. Quadricuspid semilunar valves seem to be far less common and are reported to occur nine times more frequently in pulmonic valves than aortic valves [12]. Simonds [21] could not Wnd a case of quadricuspid aortic valve in his own series of 2,000 necropsies but in a review of the literature he found two cases out of 25,666 necropsies—an incidence of 0.008%. In another series of necropsies and echocardiograms, quadricuspid aortic valve was reported in the rates of 0.008–0.013% [12]. In the available literature, pentacuspid valve is less reported than quadricuspid valve. Pentacuspid aortic valve diagnosed by transoesophageal echocardiography was reported by Cemri et al. [4] and Kamata et al. [13]. A case with a quadricuspid pulmonary valve with an accessory coronary artery was reported by Rivett and Berry [16]. Chiu et al. [5] reviewed the literature and found only one case with pentacuspid truncal valve out of 301 cases. In their report, they presented a diagram, which was useful, about the relationships of the coronary oriWce to the truncal valve according to various truncal rotations. No accessory coronary artery was observed in the present case. While quadricuspid pulmonary valves are infrequently associated with serious clinical complications, the majority of quadricuspid aortic valves are associated with clinically signiWcant dysfunction, most commonly aortic insuYciency [4]. In our case, no clinical or pathological evidence of heart failure was found, and we can speculate that the abnormal cusp formations may have been embryologically Surg Radiol Anat (2010) 32:613–615 Fig. 2 Schematic illustrations of probable mechanism of development of a normal and b abnormal semilunar valves. PV pulmonary valve, AV aortic valve. ModiWed schematic illustrations of Hirooka et al. [11] caused by the possible abnormal proliferations of pulmonary truncus of the common trunk (Fig. 2). According to our knowledge, in literature, although pentacuspid aortic valve had been reported, this is the Wrst report of a pentacuspid pulmonary valve being identiWed incidentally at an autopsy. We think that, in this case, the pentacuspid pulmonary valve is a rare congenital anomaly. Acknowledgments This study was supported by Akdeniz University Research Foundation Antalya, Turkey. The authors wish to express their deep gratitude to R. Yavuz Arican for his contributions to this work. 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