Tricuspid atresia with absent pulmonary valve and intact ventricular septum: a rare association. 2011

Thomas H Best, and Himesh V Vyas, and Robert D B Jaquiss, and Ritu Sachdeva
Division of Pediatric Cardiology, Arkansas Children's Hospital and University of Arkansas for Medical Sciences, Little Rock, AR, USA. bestthomash@uams.edu

The absence of the pulmonary valve in connection with tricuspid atresia and intact ventricular septum is a rare malformation, associated with a poor prognosis. The right ventricle is severely hypertrophied, resembling a cardiac mass protruding into the left ventricular outflow tract. We report such a case that underwent successful palliation with a Blalock-Taussig shunt followed by a superior cavopulmonary anastomosis.

UI MeSH Term Description Entries
D007231 Infant, Newborn An infant during the first 28 days after birth. Neonate,Newborns,Infants, Newborn,Neonates,Newborn,Newborn Infant,Newborn Infants
D008297 Male Males
D010166 Palliative Care Care alleviating symptoms without curing the underlying disease. (Stedman, 25th ed) Palliative Treatment,Palliative Supportive Care,Palliative Surgery,Palliative Therapy,Surgery, Palliative,Therapy, Palliative,Care, Palliative,Palliative Treatments,Supportive Care, Palliative,Treatment, Palliative,Treatments, Palliative
D011664 Pulmonary Valve A valve situated at the entrance to the pulmonary trunk from the right ventricle. Pulmonary Valves,Valve, Pulmonary,Valves, Pulmonary
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000015 Abnormalities, Multiple Congenital abnormalities that affect more than one organ or body structure. Multiple Abnormalities
D015150 Echocardiography, Doppler Measurement of intracardiac blood flow using an M-mode and/or two-dimensional (2-D) echocardiogram while simultaneously recording the spectrum of the audible Doppler signal (e.g., velocity, direction, amplitude, intensity, timing) reflected from the moving column of red blood cells. Doppler Echocardiography,Echocardiography, Continuous Doppler,Echocardiography, Two-Dimensional Doppler,2-D Doppler Echocardiography,2D Doppler Echocardiography,Continuous Doppler Echocardiography,Doppler Echocardiography, 2-D,Doppler Echocardiography, 2D,Doppler Echocardiography, Continuous,Doppler Echocardiography, Two-Dimensional,Echocardiography, 2-D Doppler,Echocardiography, 2D Doppler,Two-Dimensional Doppler Echocardiography,2 D Doppler Echocardiography,Doppler Echocardiography, 2 D,Doppler Echocardiography, Two Dimensional,Echocardiography, 2 D Doppler,Echocardiography, Two Dimensional Doppler,Two Dimensional Doppler Echocardiography
D016896 Treatment Outcome Evaluation undertaken to assess the results or consequences of management and procedures used in combating disease in order to determine the efficacy, effectiveness, safety, and practicability of these interventions in individual cases or series. Rehabilitation Outcome,Treatment Effectiveness,Clinical Effectiveness,Clinical Efficacy,Patient-Relevant Outcome,Treatment Efficacy,Effectiveness, Clinical,Effectiveness, Treatment,Efficacy, Clinical,Efficacy, Treatment,Outcome, Patient-Relevant,Outcome, Rehabilitation,Outcome, Treatment,Outcomes, Patient-Relevant,Patient Relevant Outcome,Patient-Relevant Outcomes
D017380 Hypertrophy, Right Ventricular Enlargement of the RIGHT VENTRICLE of the heart. This increase in ventricular mass is often attributed to PULMONARY HYPERTENSION and is a contributor to cardiovascular morbidity and mortality. Right Ventricular Hypertrophy,Ventricular Hypertrophy, Right,Hypertrophies, Right Ventricular,Right Ventricular Hypertrophies,Ventricular Hypertrophies, Right
D054088 Ventricular Septum The muscular structure separating the right and the left lower chambers (HEART VENTRICLES) of the heart. The ventricular septum consists of a very small membranous portion just beneath the AORTIC VALVE, and a large thick muscular portion consisting of three sections including the inlet septum, the trabecular septum, and the outlet septum. Septum, Ventricular

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