Dual Inferior Venae Cavae with Mitral Stenosis
Sandeep P Tambe, MD,
Prabhat Kumar Sinha, MD,
Ashok N Bhupali, DM
Apple Heart Institute and Research Center, Kolhapur, Maharastra, India
Sandeep P Tambe, MD, Tel: +45 50276767, Fax: +45 89498809, Email: drsptambe{at}yahoo.co.in, Anæstesi Afdeling, Århus Universitets Hospital, Skejby Sygehus, Århus 8200, Denmark.
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ABSTRACT
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A 34-year old woman with rheumatic mitral stenosis was found to have complete dual inferior venae cavae with bilateral infrarenal and suprarenal segments, on balloon mitral valvuloplasty. The bilateral, renal, and gonadal veins drained separately on the ipsilateral side. The left inferior vena cava was larger than the right, and the right inferior vena cava had an aneurysmal dilatation near its origin. The left inferior vena cava drained into the superior vena cava-right atrial junction.
Key Words: Congenital Abnormalities • Mitral Valve Stenosis • Rheumatic Heart Disease • Vascular Malformations • Vena Cava • Inferior
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INTRODUCTION
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Advances in imaging techniques have facilitated early diagnosis of various systemic venous anomalies in asymptomatic patients. A persistent left superior vena cava (SVC) with a normal right SVC is the most common. Anomalies of the inferior vena cava (IVC) and its tributaries are also well known.1 Mazzucco and colleagues2 reviewed the association of IVC anomalies with congenital cardiac anomalies. We describe a case of the rarest form of IVC anomaly associated with an acquired cardiac lesion.
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CASE REPORT
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A 34-year-old woman with acquired rheumatic mitral stenosis and dyspnea (New York Heart Association functional class III) was scheduled for balloon mitral valvuloplasty. She had been diagnosed with rheumatic mitral stenosis during her first pregnancy 8 years earlier, and treated conservatively with diuretics, digitalis, and penicillin prophylaxis for endocarditis. Cardiovascular examination revealed sinus rhythm, a loud 1st heart sound, and a mid-diastolic murmur in the mitral area. Echocardiography showed an enlarged left atrium and mitral stenosis with a valve area of 1.2 cm2. There was no associated abnormality except mild to moderate pulmonary artery hypertension with an estimated mean pulmonary artery pressure of 26 mm Hg. Chest radiography indicated mild cardiomegaly. After premedication with oral alprazolam 1 mg and intramuscular glycopyrrolate 0.2 mg, the procedure was performed under anesthesia utilizing sedation and subcutaneous local anesthetic infiltration, and monitored by electrocardiogram, O2 saturation by spirometry, and invasive blood pressure recordings via the right femoral artery. The right femoral vein was punctured, but the guidewire could not be inserted. A radiopaque dye injected through the cannula showed a narrow and tortuous course of the right IVC. After some manipulation, the guidewire was eventually passed into the vein. Under fluoroscopy, the guidewire was seen traversing the connection between the common iliac veins, crossing to the left side and reaching the right atrium (RA), high up in the left subclavian vein. Dye injected at this level and at the femoral level was seen to travel to the RA bilaterally on both sides of the spine. The left IVC drained into the SVC at the SVC-RA junction, and there was no persistent left SVC. The right IVC was smaller than the left IVC. The procedure was abandoned, and the patient was advised to have a computed tomography (CT) venogram followed by mitral valve replacement. The CT venogram showed complete duplication of the IVC, with hemiazygos continuation of the left IVC joining the SVC and draining into the RA. The right IVC was smaller in caliber than the left IVC, with aneurysmal dilatation 1.5 cm from its origin (Figure 1
). There was retroaortic communication between the 2 IVC at the infrarenal level (Figure 2). Ipsilateral renal and gonadal venous drainage was visible on the CT venogram (Figure 3). The patient refused mitral valve replacement; she was discharged with medical advice.
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Figure 1. Coronal section of a reconstructed 64-slice computed tomography scan showing the larger caliber left (a) and smaller caliber right inferior vena cava (b) with aneurysmal dilatation (d), and infrarenal communication (c) between the 2 inferior venae cavae.
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Figure 2. Axial section of a 64-slice computed tomography scan at the renal level showing the bilateral inferior venae cavae (white arrows) and left renal vein drainage (broken arrow).
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Figure 3. Sagittal section of a 64-slice computed tomography scan demonstrating the 2 left renal veins (white arrows) draining into the left inferior vena cava (dotted arrow). The smaller caliber right inferior vena cava is hidden behind the aorta (broken arrow).
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DISCUSSION
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For better understanding of the origin and variants of IVC and renal and gonadal vein anomalies, a review of IVC embryogenesis is required. Briefly, the normal IVC is composed of 4 segments: hepatic, suprarenal, renal, and infrarenal. The hepatic segment is derived from the vitelline vein. The right subcardinal vein develops into the suprarenal segment by subcardinal-hepatic anastomoses. The renal segment develops from right supra-subcardinal and post-subcardinal anastomoses. In the thoracic region, the supracardinal veins give rise to the azygos and hemiazygos veins. Progressive changes in the abdomen lead to replacement of the postcardinal veins by the subcardinal and supracardinal veins. In the pelvic region, the postcardinal veins persist as the common iliac veins.3 This complex process of IVC formation results in several variations in systemic venous return.4–6
Most patients with anomalies of systemic venous return are asymptomatic. The association of such anomalies with a symptomatic disorder leads to incidental diagnosis, as in this case. Anomalies of systemic venous return have significant clinical implications. An association of deep vein thrombosis with duplication of the IVC has been described.7 Radiological diagnosis by CT has led to early recognition of such anomalies, which has been helpful in early treatment and prevention of sudden death.5,8 Problems pertaining to the establishment of cardiopulmonary bypass and nonsurgical interventions, such as balloon mitral valvuloplasty in this case, need to be addressed further.
Prior knowledge of this anomaly would guide anesthesiologists in selecting the normal course and adequate size of the femoral vein needed for central venous access. It would also guide cardiovascular and thoracic surgeons on venous drainage in cases where femorofemoral bypass or conventional cardiopulmonary bypass needs to be instituted. Prior knowledge of this anomaly may prevent a catastrophe during bicaval cannulation for cardiac surgery, and avoid complications after the procedure, such as deep vein thrombosis and pulmonary embolism.
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REFERENCES
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- Campbell M, Deuchar DC. The left-sided superior vena cava. Br Heart J 1954;16:423–39.[Free Full Text]
- Mazzucco A, Bortolotti U, Stellin G, Gallucci V. Anomalies of the systemic venous return: a review. J Card Surg 1990;5:122–33.[Medline]
- Philips E. Embryology, normal anatomy and anomalies. In: Ferris EJ, Hipona FA, Kahn PC, Phillips E, Shapiro JH, editors. Venography of the inferior vena cava and its branches. Baltimore, MD: Williams & Wilkins, 1969:1–32.
- Bass JE, Redwine MD, Kramer LA, Huynh PT, Harris JH Jr. Spectrum of congenital anomalies of the inferior vena cava: cross-sectional imaging findings. Radiographics 2000;20:639–52.[Abstract/Free Full Text]
- Bass JE, Redwine MD, Kramer LA, Harris JH Jr. Absence of the infrarenal inferior vena cava with preservation of the suprarenal segment as revealed by CT and MR venography [Review]. AJR Am J Roentgenol 1999;172:1610–2.[Free Full Text]
- Nagashima T, Lee J, Andoh K, Itoh T, Tanohata K, Arai M, et al. Right double inferior vena cava: report of 5 cases and literature review. J Comput Assist Tomogr 2006;30:642–5.[Medline]
- Yun SS, Kim JI, Kim KH, Sung GY, Lee DS, Kim JS, et al. Deep venous thrombosis caused by congenital absence of inferior vena cava, combined with hyperhomocysteinemia. Ann Vasc Surg 2004;18:124–9.[Medline]
- Artico M, Lorenzini D, Mancini P, Gobbi P, Carloia S, David V. Radiological evidence of anatomical variation of the inferior vena cava: report of two cases. Surg Radiol Anat 2004;26:153–6.[Medline]
Asian Cardiovasc Thorac Ann 2009;
17:419-421
© 2009 by SAGE Publications
DOI: 10.1177/0218492309338099
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ABSTRACT
INTRODUCTION
