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Texas Heart Institute Journal 2003Crisscross heart, or superoinferior ventricles, is a complex and often confusing congenital anomaly. We report a heretofore unreported presentation of "isolated"...
Crisscross heart, or superoinferior ventricles, is a complex and often confusing congenital anomaly. We report a heretofore unreported presentation of "isolated" crisscross heart in situs inversus levocardia, which allows us to more clearly define the typical features of crisscross ventricles. The case of this 41-year-old woman, who had a peculiar coronary anatomy, underscores the concept that coronary artery anatomy is strictly related to the myocardial mass served. In complex congenital heart defects, development of an anterior descending artery is possible (as a primary artery, along with the circumflex and right coronary arteries) only if the ventricular septum develops properly and is aligned with the semilunar valves. We use the present case of crisscross heart to illustrate the spectrum of anomalies that can occur during formation of the cardiac apex; this spectrum ranges from a normal apex, to a diverted apex (as in dextroversion in situs solitus), to a crisscross anomaly.
Topics: Adult; Coronary Vessel Anomalies; Crisscross Heart; Female; Humans; Radiography
PubMed: 12959204
DOI: No ID Found -
Journal of Cardiovascular Echography 2016
PubMed: 28465957
DOI: 10.4103/2211-4122.178470 -
BMJ Case Reports Dec 2015A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic...
A 69-year-old man with situs inversus, levocardia and inverted great arteries developed severe dynamic left ventricular outflow tract (LVOT) obstruction with systolic anterior motion of the anterior mitral leaflet. There was no asymmetric septal hypertrophy. A possible mechanism of the LOVT obstruction in the present case may have been related to an abnormally long and bent outflow tract resulting from overriding of the right ventricle over the LVOT due to a congenital malposition of the heart. Mitral valve replacement with septal myectomy was performed in order to eliminate systolic anterior motion. The postoperative course has been excellent.
Topics: Aged; Heart Ventricles; Humans; Levocardia; Male; Tomography, X-Ray Computed; Ventricular Outflow Obstruction
PubMed: 26628311
DOI: 10.1136/bcr-2015-213030 -
British Heart Journal Jun 1960
Topics: Dextrocardia; Genetic Diseases, X-Linked; Heart Defects, Congenital; Heterotaxy Syndrome; Humans; Levocardia; Medical Records; Situs Inversus
PubMed: 13855788
DOI: 10.1136/hrt.22.3.429 -
Medical Journal, Armed Forces India Apr 1994A case of isolated levocardia with situs inversus is reported. The child had a normally formed spleen, which is uncommon in such cases.
A case of isolated levocardia with situs inversus is reported. The child had a normally formed spleen, which is uncommon in such cases.
PubMed: 28769192
DOI: 10.1016/S0377-1237(17)31023-7 -
European Journal of Human Genetics :... Sep 2015The laterality in the embryo is determined by left-right asymmetric gene expression driven by the flow of extraembryonic fluid, which is maintained by the rotary...
The laterality in the embryo is determined by left-right asymmetric gene expression driven by the flow of extraembryonic fluid, which is maintained by the rotary movement of monocilia on the nodal cells. Defects manifest by abnormal formation and arrangement of visceral organs. The genetic etiology of defects not associated with primary ciliary dyskinesia is largely unknown. In this study, we investigated the cause of situs anomalies, including heterotaxy syndrome and situs inversus totalis, in a consanguineous family. Whole-exome analysis revealed a homozygous deleterious deletion in the WDR16 gene, which segregated with the phenotype. WDR16 protein was previously proposed to play a role in cilia-related signal transduction processes; the rat Wdr16 protein was shown to be confined to cilia-possessing tissues and severe hydrocephalus was observed in the wdr16 gene knockdown zebrafish. The phenotype associated with the homozygous deletion in our patients suggests a role for WDR16 in human laterality patterning. Exome analysis is a valuable tool for molecular investigation even in cases of large deletions.
Topics: Base Sequence; Carrier Proteins; Cilia; Consanguinity; Exome; Female; Heterotaxy Syndrome; Homozygote; Humans; Hydrocephalus; Infant; Levocardia; Molecular Sequence Data; Phenotype; Sequence Analysis, DNA; Sequence Deletion
PubMed: 25469542
DOI: 10.1038/ejhg.2014.265 -
Turk Kardiyoloji Dernegi Arsivi : Turk... Sep 2014Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial...
Anatomically corrected malposition of the great arteries (ACMGA) is defined as parallel arising of aorta and main pulmonary artery (PA) roots although ventriculoarterial connection is normal. Abnormally related aorta arises from the left ventricle, while abnormally related PA arises from the right ventricle. It can be diagnosed with via echocardiography. In some cases, additional imaging modalities such as computerized tomographic angiography and magnetic resonance are required. In this article, we presented two cases of ACMGA, 5-month-old boy and 1-month-old girl. We wanted to point out the importance of differential diagnosis of other great artery anomalies from this rare pathology.
Topics: Diagnosis, Differential; Echocardiography; Female; Humans; Infant; Infant, Newborn; Levocardia; Male; Radiography; Tomography; Transposition of Great Vessels
PubMed: 25362949
DOI: 10.5543/tkda.2014.79259 -
British Heart Journal Mar 1981We have analysed the atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 hearts with isomeric atrial chambers. Of the hearts, 32 had...
We have analysed the atrioventricular junction, ventricular mass, and ventriculoarterial junction in 43 hearts with isomeric atrial chambers. Of the hearts, 32 had atrial chambers of bilateral right morphology while 11 had atrial chambers with bilateral left atrial characteristics. Among the hearts with right atrial isomerism, there were 13 biventricular hearts, all with ambiguous atrioventricular connection. Eight had a common valve and five had two atrioventricular valves. In the other 19 hearts, the atrial chambers were connected to only one ventricular chamber, 18 having double inlet ventricle through a common valve and the other having absence of the left atrioventricular connection. In these univentricular hearts, all possible types of ventricular morphology were found. The ventriculoarterial junction among these hearts with right isomerism showed great variation. In the hearts with left atrial isomerism, nine hearts had two ventricles and two were univentricular. The biventricular hearts all had ambiguous atrioventricular connection, six via a common valve and three via two valves. The two univentricular hearts both had double inlet via a common valve, one to a chamber of right ventricular type and the other to a chamber of left ventricular type. Both had rudimentary chambers of complementary pattern. The ventriculoarterial junction again showed much variation. Statistical analysis showed that pulmonary obstruction and a univentricular heart were both significantly more frequent in association with right compared with left isomerism. Significant differences were also noted in the two groups in terms of ventriculoarterial connections and infundibular morphology.
Topics: Aorta; Dextrocardia; Heart Atria; Heart Valves; Heart Ventricles; Humans; Levocardia; Myocardium; Pulmonary Artery
PubMed: 7470338
DOI: 10.1136/hrt.45.3.236 -
British Heart Journal Apr 1981Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of...
Sixty-two hearts without a patent exit from the left atrium to the ventricular mass have been studied. All had situs solitus and laevocardia. The material consisted of 32 cases with coexistent aortic atresia and 30 cases with patent aortic root. Five hearts with aortic atresia were biventricular with atrioventricular concordance and imperforate left atrioventricular valves, and 27 hearts were univentricular of right ventricular type, with absent left atrioventricular connection. The anatomy of this group was uniform, with extreme hypoplasia of all the left cardiac segments. Among the cases with patent aortic root, five were biventricular, with atrioventricular concordance and imperforate left atrioventricular valves, and 24 had absent left atrioventricular connection, 15 with univentricular heart of right ventricular type and nine with univentricular heart of left ventricular type. The final heart had double inlet univentricular of left ventricular type with an imperforate left atrioventricular valve. In this second group the aorta was larger in cases with discordant ventriculoarterial connection or in those with double outlet from the main ventricular chamber. A normal sized aorta without aortic arch obstruction was observed in nine instances. These are of great interest in terms of surgical anatomy since definitive palliation may be feasible.
Topics: Aorta; Aortic Valve; Female; Heart Atria; Heart Ventricles; Humans; Infant; Infant, Newborn; Male; Mitral Valve; Prognosis
PubMed: 7225254
DOI: 10.1136/hrt.45.4.393 -
Archives of Disease in Childhood Oct 1978In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum...
In a case of laevocardia, bronchiectasis, and paranasal sinus abnormalities, assessment on 2 occasions showed the presence of moderate immunodeficiency. Serum concentrations of IgG and IgM were low, and serum and salivary IgA was not detected. T-lymphocytes were reduced in number and cell-mediated immunity in vivo and in vitro was impaired. Opsonisation, complement system, and neutrophil functions were normal.
Topics: Bronchiectasis; Child; Female; Heart Defects, Congenital; Humans; Immunologic Deficiency Syndromes; Levocardia; Paranasal Sinuses
PubMed: 727796
DOI: 10.1136/adc.53.10.814