| HPO |
HP:0011618 |
Pulmonary situs ambiguus with bilateral morphologic right lungs |
"An abnormality of the pulmonary situs, i.e., of the sidedness of the morphological right and left lungs, in which both lungs have the morphology of a right lung." [DDD:dbrown, HPO:probinson] |
— |
HP:0011617 |
| HPO |
HP:0011619 |
Pulmonary situs ambiguus with bilateral morphologic left lungs |
"An abnormality of the pulmonary situs, i.e., of the sidedness of the morphological right and left lungs, in which both lungs have the morphology of a left lung." [DDD:dbrown] |
— |
HP:0011617 |
| HPO |
HP:0011620 |
Abnormality of abdominal situs |
"An abnormality of the abdominal situs, i.e., of the sidedness of the abdomen and its organs." [DDD:dbrown] |
— |
HP:0002012, HP:0030853 |
| HPO |
HP:0011621 |
Gerbode ventricular septal defect |
"A type of ventricular septal defect communicating directly between the left ventricle and right atrium. This is anatomically possible because the normal tricuspid valve is more apically displaced than the mitral valve." [DDD:dbrown, PMID:19561249] |
Echocardiography may visualize a jet across a small defect between left ventricle and right atrium. |
HP:0001629 |
| HPO |
HP:0011622 |
Inlet ventricular septal defect |
"A ventricular septal defect that involves the inlet of the right ventricular septum immediately inferior to the AV valve apparatus." [DDD:dbrown, PMID:17101870] |
The muscular septum is a nonplanar structure that can be divided into inlet, trabecular, and infundibular components. The inlet portion is inferioposterior to the membranous septum. It begins at the level of the atrioventricular valves and ends at their chordal attachments apically. |
HP:0001629 |
| HPO |
HP:0011623 |
Muscular ventricular septal defect |
"The trabecular septum is the largest part of the interventricular septum. It extends from the membranous septum to the apex and superiorly to the infundibular septum. A defect in the trabecular septum is called muscular VSD if the defect is completely rimmed by muscle." [DDD:dbrown, PMID:17101870] |
— |
HP:0001629 |
| HPO |
HP:0011624 |
Apical muscular ventricular septal defect |
"A muscular ventricular septal defect located at the apex of the heart." [DDD:dbrown] |
— |
HP:0011623 |
| HPO |
HP:0011625 |
Multiple muscular ventricular septal defects |
"A type of muscular ventricular septal defect characterized by the presence of multiple small defects in the ventricular septum." [PMID:22718060] |
— |
HP:0011623 |
| HPO |
HP:0011626 |
Scimitar anomaly |
"Right pulmonary venous return to the inferior vena cava." [DDD:dbrown, PMID:16638549] |
— |
HP:0010773 |
| HPO |
HP:0011627 |
Aorto-ventricular tunnel |
"Aorto-ventricular tunnel is a congenital, extracardiac channel which connects the ascending aorta above the sinutubular junction to the cavity of the left, or (less commonly) right ventricle." [PMID:17922908] |
— |
HP:0001679 |
| HPO |
HP:0011628 |
Congenital defect of the pericardium |
"A developmental defect of the pericardium with congenital onset." [DDD:dbrown] |
— |
HP:0001697 |
| HPO |
HP:0011629 |
Total absence of the pericardium |
"No pericardium around the heart, occurring as a congenital defect, not the result of a surgical pericardectomy." [DDD:dbrown] |
— |
HP:0011628 |
| HPO |
HP:0011630 |
Complete diaphragmatic absence of pericardium |
"No pericardium over the diaphragmatic surface of the heart. It is a congenital defect, not the result of a pericardectomy. Pericardium is present on other parts of the heart." [DDD:dbrown] |
— |
HP:0011628 |
| HPO |
HP:0011631 |
Complete right sided absence of pericardium |
"No pericardium is present on the righthand side of the heart. It is a congenital absence of pericardium rather than the result of a pericardectomy." [DDD:dbrown] |
— |
HP:0011628 |
| HPO |
HP:0011632 |
Partial right sided absence of pericardium |
"A congenital anomaly with lack of part of the pericardium on the righthand side of the heart." [DDD:dbrown] |
A congenital anomaly and not the result of a pericardectomy. |
HP:0011628 |
| HPO |
HP:0011633 |
Complete left sided absence of pericardium |
"A congenital anomaly with complete lack of the pericardium on the lefthand side of the heart." [DDD:dbrown] |
A congenital anomaly and not the result of a pericardectomy. |
HP:0011628 |
| HPO |
HP:0011634 |
Partial left sided absence of pericardium |
"A congenital anomaly with lack of part of the pericardium on the lefthand side of the heart." [DDD:dbrown] |
A congenital anomaly and not the result of a pericardectomy. |
HP:0011628 |
| HPO |
HP:0011635 |
Partial diaphragmatic absence of pericardium |
"Lack of a part of the pericardium over the diaphragmatic surface of the heart. It is a congenital defect, not the result of a pericardectomy. Pericardium is present on other parts of the heart." [DDD:dbrown] |
— |
HP:0011628 |
| HPO |
HP:0011636 |
Abnormal coronary artery origin |
"Isolated abnormalities of the coronary artery origins. This may be in associated with other structural heart malformations but not the patterns of complex structural heart malformations which result in abnormal course of the coronary arteries." [DDD:dbrown] |
— |
HP:0006704 |
| HPO |
HP:0011637 |
Anomalous origin of coronary artery from the pulmonary artery |
"A coronary artery begins (branches off from) the pulmonary artery rather than as normal from the root of the aorta." [DDD:dbrown, HPO:probinson] |
— |
HP:0011636 |
| HPO |
HP:0011638 |
Anomalous origin of left coronary artery from the pulmonary artery |
"Left main coronary artery begins (branches off from) the pulmonary artery rather than as normal from the root of the aorta, above the left cusp of the aortic valve." [DDD:dbrown, HPO:probinson, PMID:27358682] |
In this anomaly the function of the LMCA territory often requires extensive collateral formation from the RCA, which is often dilated. In the first month of life, physiologic pulmonary hypertension tends to preserve anterograde flow within the left coronary artery, and infants are usually asymptomatic. As pulmonary pressure drops, left-to-right shunting from the higher pressure left coronary arterial system to the lower pressure pulmonary arterial system occurs, and patients become symptomatic. Imaging findings may include markedly dilated collateral arteries and coronary veins. |
HP:0011637 |
| HPO |
HP:0011639 |
Anomalous origin of right coronary artery from the pulmonary artery |
"Right coronary artery begins (branches off from) the pulmonary artery rather than as normal from the root of the aorta, above the right cusp of the aortic valve." [DDD:dbrown, HPO:probinson] |
— |
HP:0011637 |
| HPO |
HP:0011640 |
Single coronary artery origin |
"The presence of a single coronary artery ostium from which both coronary arteries arise." [DDD:dbrown, HPO:probinson, PMID:4547578] |
— |
HP:0011636 |
| HPO |
HP:0011641 |
Coronary artery fistula |
"A congenital malformation with abnormal connection between one of the coronary arteries and a heart chamber or another blood vessel." [DDD:dbrown, HPO:probinson, PMID:20513726] |
Coronary artery fistulas are congenital malformations. This term does not apply to surgical fistulas. |
HP:0011686 |
| HPO |
HP:0011642 |
Abnormal coronary sinus morphology |
"An abnormality of the coronary sinus, which is formed by the union of the great cardiac vein and the left marginal vein and terminates in the right atrium. The coronary sinus functions to o collect deoxygenated blood from the myocardium of the heart and drain it into the right atrium." [DDD:dbrown, HPO:probinson] |
— |
HP:0005120 |