Accuracy of echocardiography in diagnosing total anomalous pulmonary venous return

Objective: Total anomalous pulmonary venous return is an uncommon cyanotic congenital heart defect. Echocardiography is the initial diagnostic tool. Complimentary non-invasive modalities like cardiac computerized tomographic angiography and cardiac magnetic resonance imaging have replaced the need for cardiac catheterization in difficult cases. This study aimed to determine the accuracy of echocardiography in diagnosing total anomalous pulmonary venous return, and to determine the factors that may decrease its sensitivity

Methods: This was a cross-sectional study conducted at the Aga Khan University Hospital Karachi, Pakistan from January 2010 to August 2016. All patients who were diagnosed with Total anomalous pulmonary venous return on echocardiography and had subsequent confirmation either on cardiac CT angiography or surgery were included. The diagnostic accuracy of echocardiography was expressed as sensitivity. Previously described taxonomy was used to define diagnostic error. Univariate and multivariate analysis were done by logistic regression OR [95 percent CI] were reported to identify factors causing the diagnostic error

Results: High diagnostic sensitivity [81 percent] was found in isolated total anomalous pulmonary venous return and low [27 percent] in heterotaxy and mixed [20 percent] varieties. Poor acoustic windows and right isomerism were found to be significant factors responsible for the diagnostic error on multivariate analysis

Conclusion: Echocardiography can diagnose isolated total anomalous pulmonary venous return with high accuracy. Use of additional modalities may be required for a complete diagnosis in cases with mixed variety, heterotaxy and poor acoustic windows

Pulmonary arteriovenous malformation in cryptogenic liver cirrhosis

The cause in 10 - 20% cases of liver cirrhosis [LC] cannot be elucidated, and are thus termed cryptogenic. Pulmonary arteriovenous malformations [PAVMs] are relatively rare, but the most common anomaly involving the pulmonary tree. Although the rare correlation between LC and PAVM is well-known, there have been no reports of PAVMs occurring in cryptogenic LC. We report a case of PAVM that occurred in cryptogenic liver cirrhosis in a 3 years old male child. The child presented with complaints of malena, hematemesis and variceal bleed. The examination revealed a child with respiratory distress, irritability, tachycardia, clubbing and abdominal distention. He was worked up for recurrent variceal bleeding secondary to portal hypertension but the oxygen saturation during hospital stay kept deteriorating. The diagnosis of hepatopulmonary syndrome as the cause of persistent hypoxemia in the absence of other cardio-pulmonary causes was then made by enhanced echocardiogram using agitated saline. He improved significantly after liver transplantation performed abroad. At a 6 months follow-up, the child was stable with no evidence of intrapulmonary shunting on repeat echo

Etiological determinants of pericardiocentesis in children

To look at etiology of pericardial effusion and association between etiology and need for pericardiocentesis. This retrospective study was conducted at the Department of Pediatrics at Aga Khan University Hospital Karachi from January 2002 - December 2010. It included children [age < 15 years] who were admitted with diagnosis of pericardial effusion. Relevant information regarding history, clinical examination, investigations and treatment were noted on a performa. Data was analyzed by using SPSS [statistical package for social science for window version 16.0]. Thirty six 36 patients admitted with moderate to large pericardial effusion were included in the study. Mean age of the patients was 79 +/- 10 months with male: female ratio of 3:1. Infections, postpericardiotomy syndrome, malignancy and connective tissue disorders were the most common causes of pericardial effusion. Seventy five percent [75%] patients required pericardial effusion drainage. Pericardial effusions secondary to infections and postpericardiotomy syndrome are more likely to require pericardiocentesis [p value 0.04]. There were two deaths in the study group which were unrelated to pericardial effusion. Infections and postpericardiotomy syndrome are the most common etiologies of pericardial effusion in our study group. Pericardial effusion secondary to infection and postpericardiotomy syndrome are more likely to require pericardiocentesis

Clinical profile and outcome of cyanotic congenital heart disease in neonates

To determine the clinical profile and assess the outcome of all neonates diagnosed with cyanotic congenital heart disease. A case series. The Aga Khan University Hospital from January 1998 to December 2000. Neonates admitted with diagnosis of cyanotic congenital heart disease were evaluated for clinical diagnosis, survival and mortality. Forty four neonates met the inclusion criteria. Eleven babies [25%] had Tetralogy of Fallot or its variants. Other malformations were d-transposition of great arteries, tricuspid valve anomalies [tricuspid atresia and Ebstein`s anomaly], hypoplastic left heart syndrome, truncus arteriosus, total anomalous pulmonary venous return and complex congenital heart disease like single ventricle. Twenty eight [63.6%] neonates survived and 16 [36.4%] expired during hospital stay. Cause of death was surgical in 2 cases and medical problems in 14 babies. Tetralogy of Fallot or variants was the commonest cyanotic heart disease in neonates with frequency of 27.27%. Majority of neonates with congenital cyanotic heart disease showed survival with appropriate management

Isolated non-compaction of left ventricular myocardium

Isolated non-compaction of the left ventricle is a distinct form of cardiomyopathy. It may be idiopathic or secondary to an identifiable disease. The condition usually presents with symptoms of cardiac failure. The present report is of a child with this disease who was diagnosed at birth and was relatively stable at 5 years of age

Reversible obstructive sleep apnea and right heart failure due to massive tonsillar hypertrophy

Obstructive sleep apnea is a relatively common but under diagnosed clinical entity in children. Adenotonsillar hypertrophy is the most common cause for upper airway obstruction in pediatric patients. If the obstruction to upper airways is not relieved, then the child can develop obstructive sleep apnea and its consequences. Treatment is simply to remove the obstruction thereby restoring patency of upper airways