Abstract
Background: Right atrial isomerism (RAI) is a heterotaxy syndrome with poor long-term outcomes, early mortality with surgical intervention [1,2], and low survival rates [3,4]. Patients with RAI have complex cardiac malformations, including total anomalous pulmonary venous return (TAPVR) [3,4], atrioventricular septal defect, ventricular hypoplasia, pulmonary stenosis/atresia, and abnormal ventriculo-arterial connections [2,3]. A number of extra–cardiac anomalies are also well defined, the most significant being asplenia [4]. We aimed to review the anatomy and outcomes of patients born in Western Australia (WA) between2003 and 2017 with RAI.
Methods: A retrospective review of patient records from2003 to 2017 within cardiology databases at Princess Margaret Hospital for Children was conducted.
Results: Nine patients (55% male), born in WA after 2003and diagnosed with RAI, were identified. In total, 77% had cardiac anomalies diagnosed antenatally; confirmed on postnatal echocardiography. All patients had atrioventricular septal defect, TAPVR and common atrioventricular valve. Pulmonary atresia was present in 66%. Asplenia was identified in eight of nine patients (88%). Surgical interventions included TAPVR repair (55%), Blalock-Taussig shunt (55%)and bidirectional cavo-pulmonary shunt (33%). Of the cohort, two of the patients have reached Fontan palliation at present. Four are deceased, with mortality occurring before the age of4 months. All deceased patients had surgical interventions; three had significant residual pulmonary venous stenosis.
Conclusion: RAI continues to be a challenging diagnosis with high mortality associated with complex cardiac anatomy and asplenism. Poor long-term outcomes with high operative risks are evident in this young WA cohort.
Methods: A retrospective review of patient records from2003 to 2017 within cardiology databases at Princess Margaret Hospital for Children was conducted.
Results: Nine patients (55% male), born in WA after 2003and diagnosed with RAI, were identified. In total, 77% had cardiac anomalies diagnosed antenatally; confirmed on postnatal echocardiography. All patients had atrioventricular septal defect, TAPVR and common atrioventricular valve. Pulmonary atresia was present in 66%. Asplenia was identified in eight of nine patients (88%). Surgical interventions included TAPVR repair (55%), Blalock-Taussig shunt (55%)and bidirectional cavo-pulmonary shunt (33%). Of the cohort, two of the patients have reached Fontan palliation at present. Four are deceased, with mortality occurring before the age of4 months. All deceased patients had surgical interventions; three had significant residual pulmonary venous stenosis.
Conclusion: RAI continues to be a challenging diagnosis with high mortality associated with complex cardiac anatomy and asplenism. Poor long-term outcomes with high operative risks are evident in this young WA cohort.
Original language | English |
---|---|
Article number | 0039 |
Pages (from-to) | S61 |
Number of pages | 1 |
Journal | Heart, Lung and Circulation |
Volume | 27 |
Issue number | Supplement 2 |
DOIs | |
Publication status | Published - 1 Jan 2018 |
Externally published | Yes |