PULMONARY ARTERY VARIANTS AND ASSOCIATED CARDIAC DEFECTS IN TETRALOGY OF FALLOT.
Objective: To see the frequency of pulmonary artery abnormalities and other associated cardiac anomalies in patients of tetralogy of Fallot (TOF).
Methodology: This was a descriptive cross-sectional study. All patients of TOF who underwent angiographic study for the diagnosis of pulmonary artery malformations and other associated cardiac defects from January 2014 to June 2016 were selected for this study. MS Excel 2013 software was used for data compilation and analysis. Percentages and mean+-SD were used to present qualitative and quantitative variables respectively.
Results: Out of 209 patients, 142 (67.9%) were male and 67 (32.1%) female. Mean age of the patients at the time of presentation was 7.65 +-5.84 years. Left pulmonary artery stenosis was the most common abnormality present in 27 (12.9%) patients, main pulmonary artery hypoplasia in 19 (9.1%), and right pulmonary artery hypoplasia in 6 (2.9%) patients. Absent pulmonary valve syndrome along with TOF was diagnosed in 2 (0.95%) patients. Associated cardiac defects were present in 82 (39.2%) patients, right aortic arch was the most common lesion presented in 36 (17.2%) patients, major aorto-pulmonary collaterals in 15 (7.2%), bilateral superior vena cava (SVC) in 10 (4.8%) and patent ductus arteriosus in 6 (2.9%) patients. Coronary artery abnormalities were diagnosed in 6 (2.9%) patients. Additional VSD was diagnosed in 4 (1.9%) patients and double aortic arch in 1(0.47%) patients. There was one patient with complete absence of IVC with azygous continuation.
Conclusion: Left pulmonary artery origin stenosis and main pulmonary artery hypoplasia were most common pulmonary artery abnormalities. Right aortic arch and major aorto-pulmonary collaterals were most common associated cardiac abnormalities in TOF patients.
Key Words: Tetralogy of Fallot, Pulmonary artery variants.
Tetralogy of Fallot (TOF) is a most common congenital cyanotic cardiac defect with the incidence ranging from 5-7% of all congenital cardiac lesions1. Large ventricular septal defect, overriding aorta, right ventricular outflow tract obstruction and right ventricular hypertrophy are typical features of TOF2-4. TOF is often associated with many other anatomical malformations e.g. small atrial septal defects, complete atrio-ventricular septal defects, absent pulmonary valve syndrome, coronary artery malformations, patent ductus arteriosus and aorto-pulmonary collaterals5-7. Pulmonary artery stenosis is the most common associated pathology with the incidence rate reaching up to 80%. Patients with TOF are usually presented with cyanosis of varying intensity. The degree of cyanosis is based upon the obstruction of flow of blood to the lungs8.
Surgical repair of TOF is the standard treatment of choice with good prognostic outcomes. But complete correction of TOF demands in-depth description of the anatomy of pulmonary vasculature and other associated cardiac defects. Angiographic studies allow more accurate diagnosis of pulmonary vasculature and associated cardiac defects along with TOF as compared to the echocardiographic study9-11. This study was conducted to see the frequency of pulmonary abnormalities and other associated cardiac anomalies in patients of TOF. Because there is a vast range of associated cardiac anomalies along with TOF and different studies have posted different prevalence rates of these anomalies so this study would help us to determine the commonest anomalies of patients of TOF belonging to Southern Punjab.
This descriptive cross-sectional study was conducted in the Department of Paediatrics Cardiology of CPE Institute of Cardiology (a tertiary care cardiac hospital). The study was retrospective in nature. We used non-randomized consecutive sampling for data collection. All patients of TOF who underwent angiographic study for the diagnosis of pulmonary artery malformations and other associated cardiac defects from January 2014 to June 2016 were selected for this study. All patients of congenital cardiac anomalies other than TOF e.g. atrial septal defect, ventricular septal defect and other complex lesions who were planned to undergo cardiac catheterization were excluded from analysis.
Sample size for this study was calculated by taking the average prevalence of 18.92% of pulmonary artery abnormalities in patients of TOF according to the study by Sheikh et al12, taking level of significance 5.0%, the sample size for this study was 191 for finite population and we included 209 patients in our analysis.
All cardiac catheterizations were done under local anesthesia. Ketamine was used as a sedative agent in younger patients. In all patients both right and left heart catheterization was done. Pulse oximetry was carried out during the procedure. Ethical approval from institutional review committee was taken for this research work. Informed consent was taken from parents of every patient before including him/her in study. MS Excel 2013 software was used for data compilation and analysis. Percentages and mean +-SD were used to present qualitative (e.g. frequency of gender, pulmonary artery variants and associated cardiac defects) and quantitative variables (age) respectively.
Out of 209 patients, 142 (67.9%) were male and 67 (32.1%) female. Mean age of the patients at the time of presentation was 7.65 +-5.84 years. About 50% patients were between 6 months to 6 years age group. Maximum age was 39 years.
Pulmonary artery variants were present in 63 (30.1%) patients. Left pulmonary artery stenosis was the most common abnormality present in 27 (12.9%) patients. Main pulmonary artery hypoplasia was present in 19 (9.1%) patients and right pulmonary artery hypoplasia in 6 (2.9%) patients. Absent pulmonary valve syndrome was present in 2 (0.95%) patients. Other pulmonary artery abnormalities are shown in table 1.
Associated cardiac defects were present in 82 (39.2%) patients, right aortic arch was the most common lesion present in 36 (17.2%) patients. Major aorto-pulmonary collaterals were present in 15 (7.2%) patients. Bilateral superior vena cava (SVC) was present in 10 (4.8%) patients. Detailed information regarding frequency of all associated cardiac defects is given in table 2.
Coronary artery abnormalities were present in 6 (2.9%) patients. Out of these, 2 (0.95%) patients have aberrant course of right coronary artery close to the right ventricular outflow tract (RVOT) and in 2 (0.95%) patients left anterior descending artery was passing close to the RVOT. In one patient, conal branch of right coronary artery (RCA) was passing close to the RVOT and in remaining one patient there was a common origin of coronary arteries. (Table 2).
Table 1: Frequency of pulmonary artery variants in patients of TOF
Pulmonary Artery Variant###Frequency (Percentage)
Left Pulmonary Artery Stenosis###27 (12.9)
Right Pulmonary Artery Stenosis###1 (0.47)
Main and Left Pulmonary Artery Stenosis###1 (0.47)
Right and Left Pulmonary Artery Stenosis###1 (0.47)
Left Pulmonary Artery Hypoplasia###2 (0.95)
Right Pulmonary Artery Hypoplasia###6 (2.9)
Main Pulmonary Artery Hypoplasia###19 (9.1)
Main and Left Pulmonary Artery Hypoplasia###2 (0.95)
Right and Left Pulmonary Artery Hypoplasia###1 (0.47)
Uniform Pulmonary Artery Hypoplasia###1 (0.47)
Absent Pulmonary Valve Syndrome###2 (0.95)
Table 2: Frequency of associated cardiac defects
Cardiac Defect###Frequency (Percentage)
Right Aortic Arch###36 (17.2)
Major Aorto-pulmonary Collateral Arteries###15 (7.2)
Bilateral SVCs###10 (4.8)
Coronary Artery Abnormality###6 (2.9)
Patent Ductus Arteriosus###6 (2.9)
Additional VSD###4 (1.9)
Double Aortic Arch###1 (0.47)
Complete absence of IVC with Azygous continuation###1 (0.47)
Tetralogy of Fallot is the most common congenital lesion that occurs in 3 out of 10,000 live births13. It results from anterior deviation of infundibular septum that cause hypoplasia of sub-pulmonary infundibulum and thus is responsible for tetrad defects e.g. overriding of the aorta, right ventricular hypertrophy, ventricular septal defect and RVOT obstruction14. TOF is unlikely to result in survival up to adulthood and is the most common congenital lesion to be encountered in adult patients after correction. Surgical repair is the only recommended treatment for TOF and have excellent prognostic results.
Long term survival has been reported to be 80% in patients with complete repair and in the absence of serious residues. These patients can live a normal life even they are able to carry out normal pregnancies15-18. But complete surgical repair of TOF require thorough knowledge about the anatomy of TOF and its associated cardiac lesions. In this study, we evaluated the common pulmonary artery malformations and other associated cardiac defects in patients of TOF undergoing angiographic analysis of these malformations before surgery.
In our study, the frequency of pulmonary artery variants was 30.1%. But in some other studies, this incidence have been reported to be 18-20%12,19-20. While some studies have found higher incidence (up to 38.8%) of pulmonary artery abnormalities in TOF patients21. In our study, the most common pulmonary malformation was left pulmonary artery origin (LPA) stenosis that was present in 12.9% patients followed by main pulmonary artery (MPA) hypoplasia which was diagnosed in 9.1% patients. Sheikh et al12 found similar results regarding incidence of left pulmonary artery stenosis, they found 10.4% incidence of LPA stenosis in patients of TOF. But in their study incidence of main pulmonary artery hypoplasia was significantly less as compared to our study. They found only 0.7% incidence of MPA hypoplasia. Saeed etal 21 found 21.4% incidence of MPA hypoplasia.
This incidence was significantly high as compared to our study. In our study, the incidence of combined MPA and LPA stenosis was 0.47 % similar to the incidence reported by many other studies. In our study, absent pulmonary valve syndrome was present in only 0.95% patients. In other studies the incidence of pulmonary valve syndrome has been reported 0.4 to 6%22-24.
In our study, associated cardiac anomalies were found in 39.2% of patients. The incidence of associated cardiac anomalies have been reported to be up to 48% in some other studies. In our study the most common associated cardiac anomaly was right aortic arch diagnosed in 36 (17.2%) patients. While in other studies the incidence of right aortic arch has been reported to be 10.0% to 29.0%8,12,21,24. In our study, bilateral SVCs present in 4.8% patients. Sheikh et al12 found 6.2% incidence of bilateral SVCs and Saeed et al found 2.8% incidence of bilateral SVCs. In our study, major aortopulmonary collaterals were present in 7.2% patients. The incidence of major aortopulmonary collaterals have been reported to be 1.1% to 28.0%8,12,21. In our study, incidence of coronary artery abnormalities was 2.9%; while other studies have reported 4.5 to 9.2% incidence of coronary artery abnormalities12,25,26.
In our study, this incidence was low as compared to other studies. In our study, patent ductus arteriosus along with TOF was diagnosed in 2.9%. Other studies, have reported 5 to 6% incidence of patent ductus arteriosus8,12,21.
Left pulmonary artery origin stenosis and main pulmonary artery hypoplasia were the most common pulmonary artery abnormalities. Right aortic arch and major aorto-pulmonary collaterals were the most common associated cardiac abnormalities in TOF patients.
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|Publication:||Journal of Postgraduate Medical Institute|
|Date:||Dec 31, 2017|
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