Double Outlet Left Ventricle, Transposition of Great Vessels and Pulmonary Stenosis

Median cleft deformities of the lower lip and mandible are very rare congenital anomalies. Our patient had median cleft of the lower lip, mandible, and the chin with tongue duplication, ankyloglossia, and cleft strap muscles with 2 neck contracture bands. This anomaly was associated with congenital heart disease transposition of great vessels, large ventricular septal defect, and severe pulmonary stenosis. Early repair was done at 6 months to improve feeding.

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Echocardiogram in d-transposition of great vessels

The American Journal of Cardiology ◽

10.1016/0002-9149(75)90882-6 ◽

1975 ◽

Vol 36 (1) ◽

pp. 121 ◽

Cited By ~ 1

Author(s):

Marc Paquet ◽

Charles E. Mullins

Keyword(s):

Transposition Of Great Vessels ◽

Great Vessels

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Left Ventricular Rhabdomyoma: A Rare Cause of Subaortic Stenosis in the Newborn Infant

PEDIATRICS ◽

10.1542/peds.46.3.464 ◽

1970 ◽

Vol 46 (3) ◽

pp. 464-468

Author(s):

Karen S. Kuehl ◽

Lowell W. Perry ◽

Roma Chandra ◽

Lewis P. Scott

Keyword(s):

Nervous System ◽

Differential Diagnosis ◽

Heart Disease ◽

Left Ventricle ◽

Tuberous Sclerosis ◽

Newborn Infant ◽

Cardiac Failure ◽

Pulmonary Stenosis ◽

Left Ventricular ◽

Subaortic Stenosis

A newborn infant with cardiac failure resulting from a rhabdomyoma of the left ventricle obstructing the aortic outflow tract is described. Mild subvalvular pulmonary stenosis was also present because of a tumor in the ventricular septum. Tuberous sclerosis occurs in at least half of infants with such tumors and was present pathologically in this child, although not apparent clinically. Rhabdomyomas must be considered in the differential diagnosis of heart disease in the newborn infant and may be associated with tuberous sclerosis even when nervous system and cutaneous signs are not present.

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Double Inlet Left Ventricle with Eisenmenger Syndrome in an Adult – A Case Report

International Journal of Medical Students ◽

10.5195/ijms.2017.173 ◽

2017 ◽

Vol 5 (1) ◽

pp. 53-56

Author(s):

Rahul Regi Abraham ◽

Rahul Regi Abraham

Keyword(s):

Congenital Heart Disease ◽

Heart Disease ◽

Left Ventricle ◽

Congenital Heart ◽

Pulmonary Stenosis ◽

Eisenmenger Syndrome ◽

Tertiary Center ◽

History Of ◽

Double Inlet Left Ventricle

Background: Patient diagnosed with double inlet left ventricle (prevalent in 5 – 10 in 100,000 newborns) complicated with Eisenmenger syndrome had a median survival age of 14 years without corrective surgery. Congenital heart disease such as this is usually treated by multiple surgeries during early childhood. A surgically uncorrected case in adults is not of common occurrence. Further, generalized itching after coming in contact with water (aquagenic pruritis) presented an interesting conundrum to treat. Case: A 29-year-old patient in India presented at a primary health care center with a history of difficulty breathing and discoloration of extremities since birth. He also gave a history of itching which commonly occurred after taking bath, hemoptysis and history of turning blue in color after birth. Patient had received no treatment besides regular phlebotomies. On examination, there was grade IV clubbing and conjunctival congestion. Cardiovascular examination revealed an enlarged heart, heaving apex beat and a pan-systolic murmur. A provisional diagnosis of a congenital cyanotic heart disease was made. Investigations revealed hemoglobin of 16.8g/dl. X–ray and electrocardiogram showed hypertrophy of the ventricles. An echocardiogram showed double inlet left ventricle with L-malposed vessels but without pulmonary stenosis. A final diagnosis of congenital heart disease; double inlet left ventricle, L-malposed vessels without pulmonary stenosis, Eisenmenger Syndrome and absolute erythrocytosis was made. Patient was advised for further management with a cardiologist in a tertiary center but the patient did not follow up. Conclusion: Unlike in high-income countries where most congenital heart diseases are detected and dealt with at birth whereas low-and middle-income nations often have to deal with cases that present much later and should often be included in the differential diagnosis. Inability to follow up cases, centers that are poorly equipped and lack of facilities for investigations, patient’s lack of medical awareness, and financial restrictions are major barriers to providing optimal treatment.

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Management of Gallbladder Empyema in a patient with Situs Inversus, Levocardia and Morbid Obesity: a Case Report.

Brazilian Journal of Case Reports ◽

10.52600/2763-583x.bjcr.2021.1.3.155-161 ◽

2021 ◽

Vol 1 (3) ◽

pp. 155-161

Author(s):

Dulcyane Ferreira de Oliveira ◽

Denis Alves Pinho ◽

Fernando Fernandes Rodrigues ◽

Luiz Fabrício Moura Marques ◽

Pedro Henrique Rosa Araújo ◽

...

Keyword(s):

Morbid Obesity ◽

Case Report ◽

Situs Inversus ◽

Surgical Intervention ◽

Rare Event ◽

Transposition Of Great Vessels ◽

Unusual Condition ◽

Abdominal Organs ◽

Left Hypochondrium ◽

Great Vessels

Situs Inversus with levocardia is an unusual condition, in which the main organs of the thorax and abdomen are located in a reverse or enantiomorphic position in relation to the usual topography. It is estimated a prevalence of 1:10000 people with some Situs Inversus condition, but Situs Inversus with Levocardia is reported in only 1:22000 cases. The presence of acute cholecystitis in patients is an extremely rare event,however, one of its possible complications, Gallbladder Empyema can develop, causing an increase in severity and the need for surgical intervention. Since the inversion of abdominal organs proper to Situs Inversus with levocardia is commonly associated with the transposition of great vessels, fatally, as described in the literature of Vesicle empyema and Situs Inversus, it only occurred in patients with dextrocardia, not yet being reported in patients with Levocardia. We report a case of a female patient with pain in the left hypochondrium with Situs Inversus Viscerum, Levocardia, Empyema of Biliary Vesicles and Morbid Obesity.

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The spectrum of disease in double outlet right ventricle

Challenging Concepts in Congenital and Acquired Heart Disease in the Young ◽

10.1093/med/9780198759447.003.0006 ◽

2020 ◽

pp. 73-84

Author(s):

Andrew Ho ◽

Tara Bharucha

Keyword(s):

Left Ventricle ◽

Ventricular Septal Defect ◽

Right Ventricle ◽

Pulmonary Stenosis ◽

Double Outlet Right Ventricle ◽

Term Infant ◽

Management Options ◽

Surgical Options ◽

Learning Points ◽

Blalock Taussig Shunt

Although often used as a diagnosis in its own right, double outlet right ventricle is more accurately merely a description of the ventriculo-arterial connection in a congenitally malformed heart, and use of the term must always be allied with further description of the anatomy. In this chapter we describe a term infant born at 3.4 kg following an antenatal diagnosis of double outlet right ventricle (DORV) with noncommitted ventricular septal defect (VSD), side-by-side great arteries and pulmonary stenosis. There was a duct-dependent pulmonary circulation in the neonatal period, and the infant underwent placement of a Blalock-Taussig shunt. Following multi-modality cardiac imaging, he underwent to complete surgical repair at 16 months of age, with VSD enlargement, tunnelled closure of the VSD to commit the left ventricle to the aorta, and augmentation of the pulmonary outflow tract. Through the chapter, we emphasise the importance of a detailed anatomical description and thorough investigation in this family of defects, in order to allow an understanding of the physiology, clinical behaviour and the management options available for individual patients. Learning points in the chapter include discussion regarding the definitions of DORV, detailed descriptions of the major anatomical groups and a synopsis of the more common surgical options.

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