González MAE, Serrano SA, Serrano RG

Language: Spanish
References: 10
Page: 677-684
PDF size: 375.36 Kb.

ABSTRACT

Pulmonary atresia with intact ventricular septum suggests a simple congenital heart malformation. However, nothing could be further from the truth. Among the morphological peculiarities of this condition, the presence of congenitally unguarded tricuspid valve orifice is rarely observed. The present paper aims at describing a series of four patients with both malformations detected in the William Soler Children’s Heart Center from 1986 through 2012. Echocardiographic diagnosis could be accurately performed in the last two patients; identification of the initial cases was established by the findings at necropsy. All patients underwent Blalock – Taussig shunt as initial palliative treatment, which was associated with balloon atrioseptostomy in two cases. Only one of the last children mentioned survived. Vital prognosis of patients suffering from this association of malformations lies in early echocardiographic detection.

REFERENCES

1. Shinebourne EA, Rigby ML, Carvalho JS. Pulmonary atresia with intact ventricular septum: from fetus to adult. Heart. 2008 ; 94 (10): 1350-7.

2. Choi YH, Seo JW, Yun YS, Kim SH, Lee HJ. Morphology of Tricuspid Valve in Pulmonary Atresia with Intact Ventricular Septum. Pediatr Cardiol. 1998 ; 19 (5): 381-9.

3. Anderson RH, Silverman NH, Zuberbuhler JR. Congenitally unguarded tricuspid orifice: its differentiation from Ebstein´s malformation in association with pulmonary atresia and intact ventricular septum. Pediatr Cardiol. 1990 ; 11 (2): 86-90.

4. González Morejón AE, Serrano Ricardo G, Díaz Ramírez F, Marcano Sanz LE. Atresia pulmonar con septo interventricular intacto. Rev Cub Pediatr [revista en Internet]. 2012 [ cited 5 Mar 2013 ] ; 84 (4): [aprox. 12p]. Available from: http://scielo.sld.cu/scielo.php?script=sci_arttext& pid=S0034-75312012000400009&lng=es&nrm=i so&tlng=es.

5. Xanthos T, Dalivigkas I, Ekmektzoglou KA. Anatomic variations of the cardiac valves and papillary muscles of the right heart. Ital J Anat Embriol. 2011 ; 116 (2): 111-26.

6. Takawira FF, Mitchell BJ, Du Plessis DJ. An unusual case of congenitally unguarded tricuspid valve orifice associated with pulmonary valve atresia and intact ventricular septum. Echocardiography. 2010 ; 27 (2): 202-4.

7. Kanjuh VI, Stevenson JE, Amplatz K, Edwards JE. Congenitally unguarded tricuspid orifice with coexistent pulmonary atresia. Circulation. 1964 ; 30: 911-7.

8. Muñoz Castellanos L, Salinas CH, Kuri Nivón M, García Arenal F. Absence of the tricuspid valve-a case report. Arch Inst Cardiol Mex. 1992 ; 62 (1): 6-7.

9. Kaneko Y, Takeda T, Yoda H, Kobayashi J, Takada N, Tsuchiya K et al. Right ventricular placation in a neonate with unguarded tricuspid valvar orifice. Ann Thorac Surg. 2009 ; 87 (1): 305-7.

10. Drant SE. The echocardiographic evaluation of pulmonary atresia with intact ventricular septum. Prog Pediatr Cardiol. 2001 ; 13 (3): 165-75.