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Asian Cardiovasc Thorac Ann 2001;9:237-239
© 2001 Asia Publishing EXchange Pte Ltd

CASE STUDY

Cardiac Malformation in Thoracopagus Twins

Türkan Tansel, MD, Fehmi Yazicioglu, MD1, Atilla Çankaya, MD1, Levent Yasar, MD1

Department of Cardiovascular Surgery Istanbul University School of Medicine Istanbul, Turkey
1 Süleymaniye Maternity Hospital
Istanbul, Turkey
For reprint information contact: Türkan Tansel, MD Tel: 90 212 635 1163 Fax: 90 212 534 2232 email: turkant{at}superonline.com Istanbul Tip Fakültesi, Kalp ve Damar Cerrahisi ABD, Capa, Istanbul 34390, Turkey.

   Abstract
TOP
 Abstract
INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
A case of thoracopagus twins with conjoined hearts and livers is described. The female twins were delivered in the 19th week of gestation. There was a common pericardial sac and the hearts were joined at the atrial and ventricular levels. Prenatally, surgical separation was considered impossible due to the fused heart, which was confirmed by the autopsy findings.


   INTRODUCTION
TOP
 Abstract
 INTRODUCTION
CASE REPORT
 DISCUSSION
 REFERENCES
 
Thoracopagus twins is a rare and fascinating congenital malformation in newborns, and it presents a major challenge to both researchers and clinicians. Although the etiology of conjoined twins is unknown, unequal and incomplete fission of the embryo at 2 to 3 weeks of gestation is considered the most likely factor.1,2 Antenatal diagnosis of conjoined twins is essential for planning optimal obstetric and perinatal management.3 As the heart anomaly is a crucial factor in survival, detection of the extent of conjoining is important.


   CASE REPORT
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
DISCUSSION
 REFERENCES
 
The mother was a 28-year-old who had one previous uncomplicated pregnancy. Prenatal ultrasound examination revealed female thoracopagus twins with single cardiac activity. The heart was grossly abnormal and inseparable. The patient requested termination of the pregnancy. Female conjoined twins were delivered vaginally in the 19th week of gestation. The fetuses were well developed with normal skeletal and brain formation. They were joined from the upper sternum to the umbilicus and faced each other. There was a single umbilical cord with one artery and one vein. At autopsy, each twin had an appropriately oriented thymus, trachea, and lungs. The lungs of both twins, which were in separate pleural cavities, had normal lobulation with 3 lobes on the right and 2 on the left (Figure 1Go). Each twin had a spleen of normal size located in the left upper quadrant. The diaphragm was common. The livers were joined but the biliary systems were separate. The gastrointestinal tracts were entirely normal in both twins.



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  		Figure 1. Superior view of the conjoined heart, great vessels, trachea, and lungs with normal lobulations. AoA = aorta of twin A, LL-A = left lung of twin A, LL-B = left lung of twin B, RL-A = right lung of twin A, RL-B = right lung of twin B.

 
There was a common pericardial sac containing conjoined hearts. The long axis of the hearts was in the sagittal plane between the twins. The hearts were joined at the atrial and ventricular levels. Four atrial appendages were present, two for each twin. The atrial complex was a common chamber caudad to the ventricles and close to the liver (Figures 2A and 2BGoGo). Each twin showed the usual atrial arrangement. The systemic and pulmonary veins in both twins drained directly into the common atrial chamber. The superior and inferior venae cavae of twin A (on the left in all figures) entered the anterior morphologically right atrium in a normal fashion. This right atrium was connected through a normal tricuspid valve to a morphologically right ventricle that communicated with the left ventricle through a ventricular septal defect. The pulmonary veins of twin A joined to form a common one that drained into the posterior morphologically left atrium. The posterior morphologically left atrium was connected through a normal mitral valve to a morphologically left ventricle. The morphologically right ventricle supported the pulmonary trunk and the morphologically left ventricle supported the aorta. The aorta was posterior and to the right of the pulmonary trunk. The aortic arch was right-sided and there was a patent right-sided ductus arteriosus. Twin B (on the left in all figures) had left-sided superior and inferior venae cavae that drained into the anterior morphologically left atrium. The left pulmonary veins of twin B drained into the anterior morphologically left atrium while the right pulmonary veins drained into the posterior morphologically right atrium. The anterior morphologically left atrium of twin B was connected to a dilated morphologically left ventricle through a normal mitral valve. Twin B had tricuspid atresia and severe hypoplasia of the right ventricle with an intact septum (Figures 3A and 3BGoGo). The aorta was anterior and to the right, while the pulmonary valve was atretic. The aortic valve of twin B had three cusps, but it was stenotic. The aortic arch was left-sided with a left-sided patent ductus arteriosus feeding the pulmonary arteries. Both of the carotid and subclavian arteries arose normally from the aortic arch in each twin. The left ventricles of both twins shared a common wall and no luminal communication was found between the two chambers.




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  		Figure 2. (A) Inferior aspect. (B) Schematic illustration of the conjoined heart showing the internal view of the common atrial chamber, systemic and pulmonary veins. ASD = atrial septal defect, CS = coronary sinus, IVC-A = inferior vena cava of twin A, IVC-B = inferior vena cava of twin B, LA-B = left atrium of twin B, LPv-B = left pulmonary veins of twin B, LV-B = left ventricle of twin B, Pv-A = pulmonary veins of twin A, Pv-B = pulmonary veins of twin B, RA-A = right atrium (morphologically left atrium) of twin A, SVC-A = superior vena cava of twin A, SVC-B = superior vena cava of twin B.

 



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  		Figure 3. (A) Anterosuperior view. (B) Schematic illustration of the conjoined heart. Ao-A = aorta of twin A, Ao-B = aorta of twin B, LA-A = morphologically left atrium of twin A, LV-A = left ventricle of twin A, LV-B = left ventricle of twin B, LPv-B = left pulmonary vein of twin B, PA-A = pulmonary artery of twin A, PA-B = pulmonary artery of twin B, PDA = patent ductus arteriosus, Pv-A = pulmonary veins of twin A, Pv-B = pulmonary veins of twin B, RA-B = morphologically right atrium of twin B, RV-A = right ventricle of twin A, RV-B = right ventricle of twin B.

 

   DISCUSSION
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
REFERENCES
 
The incidence of conjoined twins is 1 in 50,000 to 100,000 births.4,5 There is a sex predilection of 70% to 90% female and a high incidence of stillbirths.6,7 Cases are classified according to the anatomical area of conjunc-tion, and approximately 75% of all cases are thora-copagus.2,4–7 The pleural, pericardial, and peritoneal cavities are often conjoined, and the severity of the visceral malformations depends on the extent of union or incomplete separation. As in this case, the livers and hearts are often conjoined, and part of the gastrointestinal system is usually fused in cases with large areas of union. Associated cardiovascular abnormalities are found in 75% of thoracopagus twins with conjoined hearts. These abnormalities vary from a common pericardial sac to atrial and ventricular fusion with or without fusion of other organs.7 The most frequent atrial malformation in thoracopagus twins is a common atrium with a large atrial septal defect.8 The most common ventricular malformation is a single ventricle with an infundibular outlet chamber and a large ventricular septal defect.8 The great arteries are usually not fused, but they are often transposed.

Although successful surgical separation of thoracopagus twins with separate hearts has been reported, the success rate for separation of conjoined hearts is very low. Since the cardiac malformations found in thoracopagus twins are complex, surgical separation is seldom possible even when one of the twins is sacrificed. Thus, antenatal diagnosis at an early stage of pregnancy allows the option of terminating the pregnancy. This case is of particular interest because of the rarity of thoracopagus twins in the literature.


   REFERENCES
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Zimmerman AA. Embryology and anatomical considerations of conjoined twins. In: Bergsma D, editor. Conjoined twins. Birth defects original article series (Vol III, No. 1). New York: March of Dimes Birth Defects Foundation, 1967:18–27.

  2. Potter EL, Craig JM. Multiple pregnancies and conjoined twins. In: Pathology of the fetus and the infant. 3rd ed. Chicago: Year-Book, 1975:207–37.

  3. Quriroz VH, Sepulveda WH, Mercade M, Bermudez R, Fernandez R, Varela J. Prenatal ultrasonographic diagnosis of thoracopagus conjoined twins. J Perinat Med 1989; 17:297–303.[Medline]

  4. O'Neill JA, Holcomb GW, Schnaufer L, Templeton JM, Bishop HC, Ross AJ, et al. Surgical experience with thirteen conjoined twins. Ann Surg 1988;208:299–312.[Medline]

  5. Razavi-Enche F, Mulliez N, Benhaiem-Sigaux N, Gonzales M, Casasoprano A, Bloch G, et al. Cardiovascular abnormalities in thoracopagus twins: embryological interpretation and review. Early Hum Dev 1987;15: 33–44.[Medline]

  6. Marin-Padilla M, Chin AJ, Marin-Padilla TM. Cardiovascular abnormalities in thoracopagus twins. Teratology 1981;23:101–13.[Medline]

  7. Edwards WD, Hagel DR, Thompson JT, Whorton CM, Edwards JE. Conjoined thoracopagus twins. Circulation 1977;56:491–7.[Abstract/Free Full Text]

  8. Leachman RD, Latson JR, Kohler CM, McNamara DG. Cardiovascular evaluation of conjoined twins. In: Bergsma D, editor. Conjoined twins. Birth defects original article series (Vol III, No. 1). New York: March of Dimes Birth Defects Foundation, 1967:52–65.





This Article
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