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DOI: 10.1055/s-0038-1669429
Cardiothoracic Area Ratio Predicts Lethal Pulmonary Venous Obstruction in Patients with Single Ventricle and Total Anomalous Pulmonary Venous Connection
Publication History
20 December 2017
14 March 2018
Publication Date:
14 September 2018 (online)
![](https://www.thieme-connect.de/media/ajpr/201803/lookinside/thumbnails/10-1055-s-0038-1669429_170078-1.jpg)
Abstract
Background and Objectives When single ventricle (SV) is complicated with total anomalous pulmonary venous connection (TAPVC), the pulmonary vein obstruction (PVO) occurs at a high rate.
There are some patients who died from the lethal PVO (l-PVO) which needed PVO release dead due to severe desaturation within 24 hours after birth. The purpose of this study was to find a predictive marker for l-PVO during the fetal period.
Methods We enrolled 21 patients diagnosed with SV associated with TAPVC in the antenatal period. Ten patients had supracardiac, five had cardiac, five had infracardiac, and one had mixed TAPVC. We reviewed fetal echocardiography and measured cardiothoracic area ratio (CTAR) and total cardiac dimension (TCD). We divided 21 cases into l-PVO group (6) and non-l-PVO group (15) and compared the fetal echocardiography findings and postnatal prognoses between the groups.
Results CTAR at the final fetal echocardiography was 16 to 29% (median: 21) in the l-PVO group and 22 to 38% (median: 28) in the non-l-PVO group (p = 0.01). TCD/week at the final echocardiography was 0.67 to 1.0 (median: 0.77) in the l-PVO group and 0.78 to 1.2 (median: 0.96) in the non-l-PVO group (p = 0.02).
Conclusion Reduced CTAR in the antenatal period is a good predictor of l-PVO after birth.
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References
- 1 Sadiq M, Stümper O, De Giovanni JV. , et al. Management and outcome of infants and children with right atrial isomerism. Heart 1996; 75 (03) 314-319
- 2 Talwar S, Agarwal P, Choudhary SK, Airan B. Functionally univentricular heart with right atrial isomerism and mixed total anomalous pulmonary venous drainage. World J Pediatr Congenit Heart Surg 2016; 7 (03) 377-380
- 3 Khan MS, Bryant III R, Kim SH. , et al. Contemporary outcomes of surgical repair of total anomalous pulmonary venous connection in patients with heterotaxy syndrome. Ann Thorac Surg 2015; 99 (06) 2134-2139 , discussion 2139–2140
- 4 Nakata T, Fujimoto Y, Hirose K. , et al. Functional single ventricle with extracardiac total anomalous pulmonary venous connection. Eur J Cardiothorac Surg 2009; 36 (01) 49-56 , discussion 56
- 5 Hoashi T, Kagisaki K, Oda T. , et al. Long-term results of treatments for functional single ventricle associated with extracardiac type total anomalous pulmonary venous connection. Eur J Cardiothorac Surg 2013; 43 (05) 965-970
- 6 Hashmi A, Abu-Sulaiman R, McCrindle BW, Smallhorn JF, Williams WG, Freedom RM. Management and outcomes of right atrial isomerism: a 26-year experience. J Am Coll Cardiol 1998; 31 (05) 1120-1126
- 7 DiSessa TG, Emerson DS, Felker RE, Brown DL, Cartier MS, Becker JA. Anomalous systemic and pulmonary venous pathways diagnosed in utero by ultrasound. J Ultrasound Med 1990; 9 (06) 311-317
- 8 Colloridi V, Pizzuto F, Ventriglia F, Giancotti A, Pachì A, Gallo P. Prenatal echocardiographic diagnosis of right atrial isomerism. Prenat Diagn 1994; 14 (04) 299-302
- 9 Patel CR, Lane JR, Spector ML, Smith PC, Crane SS. Totally anomalous pulmonary venous connection and complex congenital heart disease: prenatal echocardiographic diagnosis and prognosis. J Ultrasound Med 2005; 24 (09) 1191-1198
- 10 Feller Printz B, Allan LD. Abnormal pulmonary venous return diagnosed prenatally by pulsed Doppler flow imaging. Ultrasound Obstet Gynecol 1997; 9 (05) 347-349
- 11 Patel CR, Lane JR, Muise KL. In utero diagnosis of obstructed supracardiac total anomalous pulmonary venous connection in a patient with right atrial isomerism and asplenia. Ultrasound Obstet Gynecol 2001; 17 (03) 268-271
- 12 Valsangiacomo ER, Hornberger LK, Barrea C, Smallhorn JF, Yoo SJ. Partial and total anomalous pulmonary venous connection in the fetus: two-dimensional and Doppler echocardiographic findings. Ultrasound Obstet Gynecol 2003; 22 (03) 257-263
- 13 Volpe P, Campobasso G, De Robertis V. , et al. Two- and four-dimensional echocardiography with B-flow imaging and spatiotemporal image correlation in prenatal diagnosis of isolated total anomalous pulmonary venous connection. Ultrasound Obstet Gynecol 2007; 30 (06) 830-837
- 14 Papa M, Camesasca C, Santoro F. , et al. Fetal echocardiography in detecting anomalous pulmonary venous connection: four false positive cases. Br Heart J 1995; 73 (04) 355-358
- 15 Allan LD, Sharland GK. The echocardiographic diagnosis of totally anomalous pulmonary venous connection in the fetus. Heart 2001; 85 (04) 433-437