Inferior Vena Cava Stenosis in Scimitar Syndrome: A Case Report

Inferior Vena Cava Stenosis in Scimitar Syndrome: A Case Report Maria Jimenez, MD, Eric Hery, MD, Nicolaas H. van Doesburg, MD, Ronald Guerin, MD, and Sheldon Spier, MD, Montreal) Quebec) Canada

The scimitar syndrome, first described by Chassinat in 1836, consists aessentially of an anomalous pulmonary vein draining whole or part of the right lung into the inferior vena cava. Associated anomalies are frequent, such as hypoplasia of the right lung, dextrocardia, malformations of the right pulmonary artery and bronchial tree, and abnormal arterial supply of the right lung (the so-called sequestration). This article describes a scimitar syndrome associated with stenosis of the inferior vena cava, whose initial diagnosis was made by two-dimensional echocardiographic Doppler color flow mapping. To our knowledge this is the first description of such an unusual association. (JAM Soc ECHo 1988;1:152-4.)

A 6-month-old boy was seen with a past history of frequent upper respiratory tract infections and bron­ chiolitis. Repeated chest roentgenograms revealed an abnormal opacity of right lower pulmonary lobe for which he was treated with antibiotics on several oc­ casions. Pulmonary auscultation revealed inspiratory crepitations in the right posterior lower lung field. There was also a I-II/VI systolic murmur at the left sternal border associated with a slight diastolic rum­ ble in the tricuspid area. The rest of the physical examination was not contributory. Results from the electrocardiogram were normal. Chest roentgenograms showed hypoplasia of the right lung with dextroposition of the heart; there was an abnormal opacity in the right lower lobe with a possible partial diaphragmatic eventration (Fig­ ure 1). An abdominal echogram demonstrated a small eventration of the diaphragm, which could not account for the whole radiographic image. A com­ puted tomography scan confirmed the right pul­ monary hypoplasia and showed in addition a pos­ sible anomaly of the vascular supply to the right lung. Two-dimensional Doppler color echocardiogra-

From the Departments of Cardiology, Pneumology, and Radi­ ology, Sainte-Justine Hospital for Children, University of Mon­ treal. Reprint requests: N. H. van Doesburg, MD, Director of Ultra­ sonic Laboratory, Division ofCardiology, Sainte-Justine Hospital, 3175, COte Sainte-Catherine, Montreal, Quebec, H3T lCS Canada.

152

phy, performed on an HP-77020 CF apparatus with a 5 MHz transducer with the subcostal approach, demonstrated an abnormal posterior vessel (7 mm in diameter) draining into the diaphragmatic portion of the inferior vena cava (IVC) (Figure 2,A). Color Doppler showed a continuous low velocity flow di­ rected toward the IVC (Figure 2, B). In addition, an IVC narrowing was seen immediately above the abnormal vessel drainage. A gradient of 8 mm Hg was measured at the site of IVC narrowing with pulsed Doppler echocardiography. The tentative diagnosis of scimitar syndrome was confirmed by cardiac catheterization, which showed a total anomalous pulmonary venous drainage of the right lung into the IVC. The suspected IVC stenosis was visualized, and there was a 9 mm Hg mean gra­ dient between IVC and right atrium with the pres­ ence of a collateral circulation at this level. Part of the right inferior lobe was supplied by an abnormal artery coming from the abdominal aorta. This se­ questered lung segment · was responsible for the roentgenographic picture. Because of the relative well-being and the young age of the infant, surgical intervention was not planned for the near future. Medical treatment con­ sisted of daily chest physiotherapy and early oral an­ tibiotic therapy at the first signs of respiratory infec­ tions. At follow-up 6 months later (age 1 year), the infant remains asymptomatic, and the pulmonary crepitations have disappeared. The cardiovascular physical examination and chest roentgenogram are unchanged.

Volume 1 Number 2 March-April 1988

Inferior vena cava stenosis

153

Figure 1 Chest roentgenogram demonstrating hypoplasia of right lung, cardiac dextropo­ sition, and opacification of right lower lung field.

Figure 2 A, Abnormal vessel (APVR) (7 I_I1l11 in diameter) draining into diaphragmatic portion of inferior vena cava (IVC). IVC narrowing immediately above anomalous vessel. RA, Right atrium.

DISCUSSION

The scimitar syndrome 1 is a well known although rare entity. Associated anomalies2 are present in 25% of the cases3 and consist mainly of atrial septal defect, patent ductus arteriosus, coarctation of the aorta, ventricular septal defect, and tetralogy of Fallot. As­ sociated stenosis of the IVC is described for the first time in this present case.

In the classical syndrome the clinical picture is vari­ able. There may be few or no symptoms. The di­ agnosis is often suspected by the demonstration of an abnormal site of pulmonary venous drainage (the scimitar sign) on the chest roentgenogram or of an abnormal lung opacity caused by the sequestration of a pulmonary lobe; the right lung is often hypo­ plastic. The computed tomography scan4 can be useful in

Journal of the American Society of Echocardiography

154 Jimenez et a!.

Figure 2 B, Same figure with color Doppler. Flow in abnormal vessel is of low velocity (69 em/sec) and directed toward transducer and IVC (yellow). Flow acceleration is noted at level ofiVC stenosis (IVC STEN.), where there is also aliasing (ltWsaic ofcolors).

delineating with greater precision the anomalous ve­ nous drainage, but final diagnosis is usually made by cineangiography. Two-dimensional Doppler color echocardiography allows a visualization of the anom­ alous pulmonary vein, 5•6 its course, and detection of associated anomalies (stenosis ofthe IVC in the pres­ ent case). This noninvasive technique is easily per­ formed and should provide fast and precise anatomic information on the anomalous pulmonary veins m patients with suspected scimitar syndrome.

2. 3. 4. 5.

6.

REFERENCES l. Chassinat R. Observation d'anomalies anatomiques remarqua­

bles de l'appareil circulatoire avec hepatocele congenitale,

n'ayant donne lieu pendant Ia vie aaucun symptome particulier. Arch Gen Med Paris 1836;11:80. Farnsworth AE, Ankeney JL. The spectrum of the scimitar syndrom,e. J Thorac Cardiovasc Surg 1974;68:37-42. Gikonyo DK, Tandon R, Lucas RV Jr, Edwards JE. Scimitar syndrome in neonates: report of four cases and review of lit­ erature. Pediatr Cardiol1986;6:193-7. Godwin JD, Tarver RD. Scimitar syndrome: four new cases examined with CT. Radiology 1986;159:15-20. Satomi G, T akao A, Momma K, et a!. Detection ofthe drainage site in anomalous pulmonary venous connection by two­ dimensional Doppler color flow-mapping echocardiography. Heart Vessels 1986;2:41-4. Vitarelli A, Scapato A, Sanguigni V, Caminiti MC. Evaluation of total anomalous pulmonary venous drainage with cross­ sectional colour flow Doppler echocardiography. Eur Heart J 1986;7: 190-5.