Echo Quiz |
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ECHO OF THE MONTH
A Child with Cyanosis
Munesh Tomar, S. Radhakrishnan, Savitri Shrivastava
Department of Pediatrics & Congenital Heart Diseases
Escorts Heart Institute & Research Centre,New Delhi 110025 |
A CHILD WITH CYANOSIS
Three-year old male child presented with cyanosis, no CHF,
no significant murmur on examination. Figures given are
Subcostal sagittal view with color flow mapping and contrast
echocardiography (first cardiac cycle) with agitated saline
given from right brachial vein in apical four-chamber view. |
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Figure 1a: Subcostal sagittal view with color flow mapping showing flow
of right superior vena cava (SVC) to left atrium. |
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ANSWER
Left atrial drainage of the right SVC is a rare malformation that
typically manifests itself as unexplained cyanosis and clubbing
in patients who do not have any other signs of a heart defect 1. On
reviewing the literature we could find 23 cases published in
English1-4 .The right upper lobe pulmonary veins usually drained
into the right SVC5. Van Praagh and associates postulate that this
represents an anomaly similar to sinus venosus atrial septal
defect of the superior vena caval type in association with atresia
of the superior vena caval orifice3. Pressure differences between
the left and right atria determine the preferential flow of blood
and if the superior vena caval blood flow to the left atrium |
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Figure 1b: After contrast injection, there is direct filling (with first
cardiac cycle) of left atrium and left ventricle without any opacification
of right atrium due to anomalous connection of right superior vena cava
to left atrium. |
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REFERENCES
1. Van Praagh S, Geva T, Lock JE, et al. Biatrial or left atrial drainage of the right superior
vena cava: Anatomic, morphogenetic, and surgical considerations report of three new
cases and literature review. Pediatr Cardiol 2003; 24:350–363.
2. Wood P. Diseases of the Heart and Circulation. 2nd ed. London: Eyre and Spottiswood,
1957; 457–458.
3. Shapiro EP, Al-Sadir J, Campbell NPS, et al. Drainage of the right superior vena cava into
both atria. Circulation 1981; 63:712–717.
4. Alday LE, Maisuls H, De Rossi R. Right superior caval vein draining into the left atrium—
diagnosis by color flow mapping. Cardiol Young 1995; 5:345–349.
5. Akalin H, Uysalel A, Ozyurda U, et al. The triad of persistent left superior vena cava
connected to the coronary sinus, right superior vena cava draining into the left atrium, and
atrial septal defect: Report of a successful operation for a rare anomaly. J Thorac
Cardiovasc Surg 1987; 94:151–153. |
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Correspondence: Munesh Tomar, Department of Pediatrics & Congenital Heart Diseases Escorts Heart Institute & Research Centre,New Delhi 110025
Email: muneshtomar@yahoo.co.in |
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Indian Heart J. 2009; 61:208 |
