Congenital heart diseases in noonan's syndrome - mitral valve anomalies: important cause of left ventricular outflow tract obstruction

Background: Noonan's Syndrome [NS] is a common genetic disorder associated with congenital heart disease. It is an autosomal dominant. Cardiac defects are found in 50-60% of cases of this syndrome. Ventricular outflow obstruction in the form of pulmonary valvular stenosis and hypertrophic cardiomyopathy [HOCM] is the most common congenital heart condition. Apart from HOCM, mitral valve anomalies are also associated with left ventricular outflow obstruction

Objective: To identify congenital heart diseases associated with Noonan's syndrome

Design: A Retrospective Study

Setting: Pediatric Cardiac Outpatient Service, Bahrain Defence Force Hospital, Bahrain

Method: All patients with confirmed Noonan's syndrome from 2005 to 2018 were included in the study. Cardiac evaluation was performed by chest X-ray, ECG, 2D echocardiography and cardiac catheterization. Personal characteristics were documented: age at diagnosis, sex and consanguinity. Morphologic types of congenital heart diseases [CHD] and outcomes were analyzed

Result: Twenty-nine children with Noonan's syndrome; 15 [52%] males and 14 [48%] females were included in the study from 2005 to 2018. Pulmonary valvular stenosis, 21 [72%], was the most common cardiac anomaly followed by HOCM, 5 [17%]. Mitral valve tissue was found in two [7%] patients. Severe obstruction to the left ventricular outflow by accessory mitral tissue was identified in one [3%] patient which required surgery

Conclusion: Cardiac defects are common in NS. Left outflow tract obstruction due to accessory mitral valve tissue is rare; however, it is a significant anomaly and if not specifically looked for on echocardiography, it could be missed

Systemic to pulmonary shunting in 93 cyanotic neonates

Background: Systemic to pulmonary shunting is still the main palliation option in many neonates with cyanotic heart disease

Design: A Retrospective Study

Setting: The Mohammed bin Khalifa bin Salman Al-Khalifa Cardiac Center, Bahrain Defence Force Hospital, Bahrain

Method: All infants from January 1995 to December 2008 who had undergone systemic to pulmonary shunting procedures were included in the study

Result: Ninety-three infants underwent 108 shunt type procedures, 39 females and 54 males. One hundred five Modified Blalock-Taussig Shunts [MBTS] and three classic Blalock-Taussig shunts were performed. Tetralogy of Fallot/Double Outlet Right Ventricle [DORV] with right ventricular outflow tract obstruction was the most common diagnosis, 51 [55%]. Early postoperative mortality was 1 [1%]. Late postoperative mortality was 4 [4%]; sepsis was the major cause. Early [<1 month postoperatively] shunt failure/occlusion occurred in 6 [6%] patients. Under 14 days of age at the time of shunting and shunt size of 5mm [18% versus 4.6%] were risk factors for early shunt occlusion/failure. Eight [8%] patients required late shunt revision at two months to 5 years post initial shunting. Fifty-one patients had undergone final corrective surgery

Conclusion: MBTS shunting provides effective and safe palliation in small infants with complex cyanotic heart disease. Early shunting [<14 days of age], and 5mm shunt size are additional risk factors for early shunt failure

Acute myocarditis and cardiac magnetic resonance imaging

A thirty-two-year-old female presented with acute myocarditis which was confirmed with Cardiac Magnetic Resonance Imaging [CMRI] where endomyocardial biopsy sampling was not feasible. The patient was managed initially with dual anti-platelet therapy and heparin; the patient was discharged home after four days on 75 mg aspirin

Large atrial septal defects in adolescents and adults: a high incidence of sinus venosus type defects presenting late

Background: Isolated Atrial Septal Defect [ASD] is a common congenital heart disease that could present at any age. It is the most common form of heart disease presenting in adolescents and adults

Objective: To evaluate adolescents and adults presenting late with significant ASD's and their presentation, management and short-term outcomes

Design: A Retrospective Study

Setting: Mohammed Bin Khalifa bin Salman Al-Khalifa Cardiac Center, Bahrain

Method: A retrospective study was performed. Adolescents/adults more than 14 years of age with significant ASD referred to a tertiary cardiology unit over a three-year period were reviewed from 1 March 2012 to 31 March 2015. The data was obtained from a computerized database

Result: Thirty-two patients presented with large ASD. Twenty-five [78.1%] were females. The mean age was 29.4 years. The majority were diagnosed as an incidental finding of murmur during routine examination. A high number of Sinus Venosus Defects in the adolescent/adult group [22% compared to 7% in the pediatric group: P=0.01] was found, which were more common in males [42% compared to 17%: P=0.128]. Nine [28.1%] underwent successful device closure. Twenty three [71%] patients were referred for surgical correction. There was no mortality

Conclusion: Significant ASD is often undiagnosed until adolescence or adulthood. If not promptly diagnosed and managed, it is likely to present with symptoms and signs of pulmonary overload later in life. Sinus Venosus ASD's should be suspected in male adolescents and adults with significant ASD's in Bahrain and surgical closure is often required