HPO
Db Link Name Definition Comment Is a
HPO HP:0011543 Superior-inferior ventricles without criss-cross atrioventricular valves HP:0011534
HPO HP:0011544 L-looping of the right ventricle HP:0011534
HPO HP:0011545 Abnormal connection of the cardiac segments "A deviance in the normal connections between two cardiac segements." [PMID:24876921] HP:0001627
HPO HP:0011546 Abnormal atrioventricular connection "An abnormality of the circulatory connection between atria and ventricles." [DDD:dbrown] HP:0011545
HPO HP:0011547 Absent left sided atrioventricular connection "A defect where there is no connection between the left atrium and left ventricle." [PMID:7104116] HP:0011546
HPO HP:0011548 Absent right sided atrioventricular connection "A defect where there is no connection between the right atrium and right ventricle." [PMID:7104116] HP:0011546
HPO HP:0011549 Univentricular heart with absent left sided atrioventricular connection HP:0011547
HPO HP:0011550 Biventricular heart with straddling right sided atrioventricular valve and absent left sided atrioventricular connection HP:0011547
HPO HP:0011551 Right sided atrium to left ventricle and absent left sided atrioventricular connection HP:0011547
HPO HP:0011552 Ambiguous atrioventricular connection "With left or right cardiac isomerism in a biventricular, the atrioventricular connections are perforce ambiguous, in that one of the connections is concordant (e.g., right-sided morphologic right atrium connected to a morphologic right ventricle) and one of the connections is discordant (e.g., left-sided morphologic right atrium connected to a morphologic left ventricle)." [DDD:dbrown, HPO:probinson] HP:0011546
HPO HP:0011553 Discordant atrioventricular connection "Connection of the right atrium to the left ventricle and of the left atrium to the right ventricle in a biventricular heart." [DDD:dbrown, HPO:probinson, PMID:23804932] HP:0011546
HPO HP:0011554 Double inlet atrioventricular connection "The condition in which both atria are joined to a single ventricle each by its own atrioventricular valve." [DDD:dbrown, HPO:probinson] HP:0011546
HPO HP:0011555 Double inlet left ventricle "The condition in which both atria are joined to the left ventricle each by its own atrioventricular valve. Usually there is a hypoplastic right ventricle, which may be on the opposite side of the heart as usual." [DDD:dbrown, HPO:probinson] HP:0001750, HP:0011554
HPO HP:0011556 Double inlet right ventricle "The condition in which both atria are joined to the right ventricle each by its own atrioventricular valve. Usually, the left ventricle is hypoplastic." [DDD:dbrown, HPO:probinson] HP:0001750, HP:0011554
HPO HP:0011557 Double inlet to single ventricle of indeterminate morphology "The condition in which both atria are joined to a single ventricle each by its own atrioventricular valve. The morphology of this ventricle does not allow one to determine if it corresponds to the left or right ventricle." [DDD:dbrown, HPO:probinson] HP:0001750, HP:0011554
HPO HP:0011558 Double inlet to single ventricle with common atrioventricular orifice HP:0011557
HPO HP:0011559 Double inlet to single ventricle with two atrioventricular valves HP:0011557
HPO HP:0011560 Mitral atresia "A congenital defect with failure to open of the mitral valve orifice." [DDD:dbrown] HP:0001633, HP:0011546
HPO HP:0011561 Overriding atrioventricular valve "An atrioventricular valve that empties into both ventricles. The valve overrides the interventricular septum above a ventricular septum defect." [DDD:dbrown, HPO:probinson, PMID:7295418] Straddling and overriding atrioventricular valves usually occur in combination. HP:0011546
HPO HP:0011562 Straddling atrioventricular valve "Anomalous insertion of the chordae tendinae or papillary muscles into the contralateral ventricle in the presence of a ventricular septum defect." [DDD:dbrown, HPO:probinson, PMID:7295418] Straddling and overriding atrioventricular valves usually occur in combination. HP:0011546
HPO HP:0011563 Abnormal ventriculoarterial connection "An abnormality of the circulatory connection between the ventricles and the pulmonary artery and aorta." [PMID:15227248, PMID:18805185, PMID:24750982] The cardiac malformations with abnormal ventriculo-arterial connections in situs solitus and atrio-ventricular concordance include the tetralogy of Fallot as well as different forms of double-outlet right ventricle and transposition of the great arteries. HP:0011545
HPO HP:0011564 Mitral valve arcade "Anomalous mitral valve arcade is diagnosed based on the following features (1) An adequately sized mitral valve orifice; (2) short, thick, and poorly differentiated chordae with direct union of the papillary muscles to the anterior leaflet; (3) narrow or nearly nonexistent spaces between the abnormal chordae; and (4) greater differentiation of the chordae attached to the posterior papillary muscle." [DDD:dbrown, PMID:20404264] HP:0001633
HPO HP:0011565 Common atrium "Complete absence of the interatrial septum with common atrioventricular valve and two atrioventricular connections." [DDD:dbrown, HPO:probinson] HP:0005120
HPO HP:0011566 Cor triatriatum dexter "A congenital anomaly with partitioning of the right atrium to form a triatrial heart caused by persistence of the right valve of the sinus venosus. Typically, the right atrial partition is due to exaggerated fetal eustachian and thebesian valves, which together form an incomplete septum across the lower part of the atrium. This septum may range from a reticulum to a substantial sheet of tissue." [DDD:dbrown, HPO:probinson, PMID:17948095] Cor triatriatum dexter is a Latin phrase meaning that the heart (cor) has three atria (triatriatum) whereby the 'third' atrium is on the right (dexter) side. HP:0010774
HPO HP:0011567 Sinus venosus atrial septal defect "An interatrial communication caused by a deficiency of the common wall between the superior vena cava (SVC) and the right-sided pulmonary veins. SVASD is commonly associated with anomalous pulmonary venous connection (APVC) of some or all of the pulmonary veins, which produces additional left-to-right shunting." [DDD:dbrown, HPO:probinson, PMID:16172274] HP:0001631