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| CASE REPORT |
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| Year : 2019 | Volume
: 5
| Issue : 2 | Page : 125-127 |
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Snowman heart
Sachin Sondhi1, PC Negi1, Sanjeev Asotra1, Ayushi Mehta2
1 Department of Cardiology, IGMC, Shimla, Himachal Pradesh, India
2 Department of Anaesthesia, IGMC, Shimla, Himachal Pradesh, India
| Date of Submission | 10-Apr-2019 |
| Date of Decision | 13-May-2019 |
| Date of Acceptance | 25-May-2019 |
| Date of Web Publication | 19-Aug-2019 |
Correspondence Address:
Dr. Sachin Sondhi
Department of Cardiology, IGMC, Shimla, Himachal Pradesh
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/jpcs.jpcs_27_19
Total anomalous pulmonary venous connection (TAPVC) consists of abnormality of blood flow in which all pulmonary veins drain into systemic veins or into right atrium with or without pulmonary venous obstruction. Systemic and pulmonary venous blood mix in right atrium. An atrial septal defect is required for survival. We report a case of supracardiac TAPVC and discussed about its diagnostic and management approach.
Keywords: Common pulmonary vein, figure of 8 appearance, snowman heart, supracardiac total anomalous pulmonary venous connection, vertical vein
How to cite this article:
Sondhi S, Negi P C, Asotra S, Mehta A. Snowman heart. J Pract Cardiovasc Sci 2019;5:125-7 |
| Description | |  |
A 26-year-old female presented with complaints of class 2 dyspnea and exertional palpitations for the last 3 years. On cardiovascular examination, she had mild cyanosis with a of 86% in all the limbs, no conjunctival suffusion, grade 2 clubbing, raised jugular venous pressure (prominent v wave and y descent), right ventricular (RV) type apex, grade 3 parasternal heave, a wide split second heart sound with loud pulmonary component, grade 3/6 ejection systolic murmur over left upper sternal border, grade 2/6 pansystolic murmur, and diastolic flow rumble over the left lower sternal border. The electrocardiogram (ECG) showed right axis deviation (RAD) and rsR' pattern. The chest X-ray showed cardiomegaly, RV type apex, dilated right descending pulmonary artery, increased pulmonary vascularity, end-on vessels, and snowman sign (figure of eight appearances) [Figure 1]. Based on these findings, the possibility of a supracardiac total anomalous pulmonary venous connection (TAPVC) was kept. | Figure 1: Chest X-ray showing figure of 8 or snowman heart. The figure of 8 is formed by ascending vertical vein (horizontal yellow arrow on the left side) and dilated superior vena cava (horizontal white arrow on the right side). Also note, cardiomegaly with the right ventricular type of apex, increased pulmonary vascularity, dilated right descending pulmonary artery (curved white arrow on the right side), and end-on vessels (star-shaped yellow mark on the left) suggestive of pulmonary plethora
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The transthoracic echocardiography in subcostal 4 chamber revealed dilated right atrium (RA), dilated RV, large atrial septal defect (ASD), and all pulmonary veins draining into the common pulmonary vein (CPV) which was located posterior and superior to the left atrium [Figure 2]. The pulmonary flow velocity (2.11 cm/s) was more than aortic flow velocity (1 cm/s), and there was moderate tricuspid regurgitation with a gradient of 59 mmHg. The CPV gave rise to a vertical vein (VV), which was seen on suprasternal long axis view, and it was draining into superior vena cava (SVC) through a left innominate vein [Figure 3] and it showed phasic low-velocity flow. Hence, the final diagnosis of supracardiac TAPVC was made, and the patient was sent for a cardiac catheterization study. | Figure 2: Transthoracic echocardiogram, the subcostal view showing dilated right atrium and right ventricle with large atrial septal defect. Also note the absence of the pulmonary venous drainage to the left atrium. They form a common pulmonary vein ((blue arrow). The color flow on the right side shows right to left shunt across atrial septal defect and flow in common pulmonary vein.
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 | Figure 3: Transthoracic echocardiogram, the suprasternal view showing vertical vein (horizontal blue arrow) draining into a left innominate vein The left innominate vein drained into the right superior vena cava. The right side of figure on color flow showed torrential flow in the vertical vein (curved blue arrow). AO: Aorta, PA: Pulmonary artery.
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TAPVC is a rare congenital heart disease, representing nearly 1.5%–2% of all cardiovascular anomalies.[1] In TAPVC, all the pulmonary veins join to form the common confluence known as the CPV and this CPV ultimately drains into the RA through various routes:
- Supracardiac TAPVC (CPV > VV > LIV > SVC > RA)
- Cardiac (CPV > CS [coronary sinus] >RA)
- Infracardiac (CPV > VV > PV [portal vein] >IVC >RA)
- Mixed type.
In supracardiac TAPVC, which is a most common type of TAPVC, there is mixing of deoxygenated blood in SVC with oxygenated blood from pulmonary veins; this mixed blood enters pulmonary artery through right-sided chambers and into the left atrium through the large ASD.[2] Symptoms of failure to thrive and recurrent respiratory tract infections and mild cyanosis were present in children. Sometimes, the supracardiac TAPVC is obstructed between the left pulmonary artery and left bronchus which is known as a bronchopulmonary vice, and this may lead to pulmonary edema in infants.[3] Clinical findings in adults are the same as that of an ASD, a wide fixed split of second heart sound and ejection flow murmur over the left upper sternal border. If the shunt is large, then a diastolic flow rumble over the left lower sternal area and RV third heart sound can be heard. The ECG will show right bundle branch block (RBBB) (rsR') and RAD, and the chest x-ray reveals a figure of 8 appearance (snowman sign). The diagnosis is confirmed by echocardiography[4] followed by computed tomography angiography to look anatomy and course of pulmonary veins.[5] During cardiac catheterization, oxymetry data will help in localizing the drainage site of anomalous pulmonary veins. In supracardiac TAPVC, there is a step up in oxygen saturation at SVC level. Adequate mixing in the RA is the rule. Thus, oxygen saturation RV, pulmonary artery, left atrium, left ventricle, and aorta are expected to be equal to that in the RA. During cardiac catheterization shunting (Qp: Qs) and pulmonary vascular resistance (PVR) is calculated. Sometimes in borderline cases, pulmonary artery hypertension (PAH) reversibility testing is required. Performing selective pulmonary arteriography and watching the levophase for pulmonary venous return may image the connections. If the anomalous connection is entered, direct injection of radiographic contrast dye will delineate the anatomy. In supracardiac TAPVC, corrective surgery is performed and the basis of this correction is the creation of large side to side anastomosis between left atrium and common pulmonary venous confluence. Subsequently, the ASD is closed with a patch, and the site of anomalous connection is ligated.[6],[7]
The prognosis of TAPVC is influenced by the size of interatrial communication and the presence of obstructive lesions in anomalous venous pathways. The status of the pulmonary vascular bed, by determining the magnitude of PBF, also plays a significant role in prognosis. In a survey study of TAPVC of all types, 50% were dead at the age of 3 months and 80% had died by 1 year of age.[8] The survival in our patient up to the age of 26 years was explained by the presence of a large ASD.
Learning points
- In patients of clinically suspected ASD, if there is cyanosis, always look for anomalous pulmonary venous drainage on echocardiography
- The chest X-ray has a typical snowman sign (figure of 8) in unobstructed supracardiac TAPVC
- On echocardiography always look for draining site for a CPV in TAPVC. The most common site is right-sided SVC through a VV. In supracardiac TAPVC, suprasternal view is necessary for delineating the course of the VV
- During cardiac catheterization, performing selective pulmonary arteriography and watching the levophase for pulmonary venous return may image the connections.
Financial support and sponsorship
Nil.
Conflicts of interest
There are no conflicts of interest.
| References | | |
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| 2. | Geva T, van Praagh S. Anomalies of the pulmonary veins. Allen HD, Driscoll DJ, Shaddy RE, Feltes TF, Moss and Adams' heart disease in infants, children, and adolescents: including the fetus and young adult. 7 th ed. Philadelphia: Lippincott Williams & Wilkins; 2008. p. 761-92. |
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| 4. | Goswami KC, Shrivastava S, Saxena A, Dev V. Echocardiographic diagnosis of total anomalous pulmonary venous connection. Am Heart J 1993;126:433-40. |
| 5. | Liu J, Wu Q, Xu Y, Bai Y, Liu Z, Li H, et al. Role of MDCT angiography in the preoperative evaluation of anomalous pulmonary venous connection associated with complex cardiac abnormality. Eur J Radiol 2012;81:1050-6. |
| 6. | Vicente WV, Dias-da-Silva PS, Vicente Lde M, Bassetto S, Romano MM, Ferreira CA, et al. Surgical correction of total anomalous pulmonary venous drainage in an adult. Arq Bras Cardiol 2006;87:e172-5. |
| 7. | Wetzel U, Scholtz W, Bogunovic N, Körfer J, Haas NA, Blanz U, et al. Successful correction of a total anomalous venous connection in a 63-year-old male – Case report and review of the literature. Congenit Heart Dis 2010;5:470-5. |
| 8. | Keith JD, Rowe RD, Vlad P, O'hanley JH. Complete anomalous pulmonary venous drainage. Am J Med 1954;16:23-38. |
[Figure 1], [Figure 2], [Figure 3]
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