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Transcript
Artial Septal Defect J.A. Moutiris, MD, MSc, PhD, FESC Department of Cardiology Paphos General Hospital Atrial Septal Defect By: Aspasia Spyrou(1), Ifigeneia Charalambous(2), Joseph Moutiris(3), Christos Kontos(3), Ariadni Stylianou(3), Ioannis Michaelides(3),Petros Mavrommatis(3) (1) final year medical student, Royal College of Surgeons in Ireland (2) final year medical student, University of Ioannina, Greece (3) cardiologists, cardiology dept, paphos general hospital Simple definition • ASD is a hole in the wall (septum) that separates the two upper chambers (atria) of the heart. The defect allows blood to flow from one atrium to the other, usually from the left side to the right side. This causes extra blood flow: • In the right atrium • In the right ventricle • To the lungs Incidence • • • • Second commonest congenital heart defect. 5-10% of all cases of CHD,22-40% in adults. 2:1 female to male predominance. More than 3,000 children are affected in the U.S every year. • Ostium secundum type more common 60-70%. • Ostium primum 30%. Etiology • foramen ovale – a large oval opening in the septum secundum. When infant takes first breath, higher pressure in left atrium slams septum primes against foramen ovale. Over time, the edges of the foramen ovale fuse with the septum primum and the adult remnant is the fossa ovalis. Ostium Secundum septum primum continued.. • septum primum – first part of the interatrial septum, grows from the roof of the atrium towards the floor. It is thin, and doesn’t quite reach the floor. The gap that persists is the ostium primum. When it does reach the floor it perforates, and the holes coalesce into the ostium secundum. The septum primum forms the flap that covers the foramen ovale. • septum secundum – A second ridge of tissue that descends from the roof of the atrium as the osteum secundum is coalescing. The foramen ovale forms in the septum secundum. In a nutshell… • Two main types of ASD • Foramen ovale if the defect is in the ostium secundum. • Or an ostium primum defect which results in:an inter-atrial communication, abnormal atrioventricular junction and abnormal atrioventricular valves-hallmark is a trileaflet valve. Other Congenital heart defects • Fallot’s tetralogy includes: ventricular septal defect, overriding of the aorta, pulmonary hypertension and right ventricular hypertrophy. • Eisenmerger’s syndrome=VSD with pulmonary hypertension. • Valve atresias • Coarctation of the aorta • Transposition of the great vessels ASD in Ellis-van Creveld Dysplasia • Skeletal dysplasia • Affetcs 1:60,000 births. • Commonest amongst Ammish people of Pensylavania and in Western Australia. • Autosomal recessive • Recent studies have shown 2 mutations on chromosome 4 (4p16). Clinical Picture of EVD • EVD is characterized by dental abnormalities, natal and small teeth and pseudocleft. • Shortening of lower limbs, polydactyly, hypoplastic finger nails, progressive genu valgus and knock-knees. • 60% have congenital heart defects most commonly ASD. • Genitourinary anomalies • Hydrocephalus. • Note that referral to a cardiologist is highly recommended. In summary Signs and symptoms • Systolic ejection murmur at left lower sternal border. • Widely split S2 • Loud S1 • Small defects can be asymptomatic. • Large defects may cause dyspnea, poor feeding in children, poor growth, recurrent respiratory infections and TIAs in unusually young adults. Diagnosis • • • • • • Clinical Examination CXR ECG Echocardiogram with Doppler Trans esophageal echo with saline Cardiac catheterization The CXR and the ECG The ECG of a patient showing right heart hypertrophy: peaked P and R waves as well as deep S on V1 and V6. The CXR of a patient demonstrating significant cardiomegalyand pulmonary congestion due to an ASD. Transoesophageal echocardiogram Transoesophagial echocardiogram with saline solution Patent foramen ovale Antenatal screening for ASD • Antenatal diagnoses rates are poor and this is of great importance when counseling parents with an apparently normal fetal scan. • Trisomy 21 or Down’s syndrome is strongly associated with congenital heart disease with primum ASD being the commonest one. Soft markers on fetal ultrasound such as endocardial cushions can be misleading and therefore in cases of suspicion for Down’s syndrome amniocentesis or chorionic villus sampling should be advised. Facts on Newborn examination • A meta-analysis was carried out in the U.K, in 2005 and included 100,000 live born infants. The study concluded: • 121 infants with life threatening congenital heart disease were undiagnosed at screening of whom 82 and 83 were diagnosed by pulse oximetry and echocardiography respectively. more.. • Only 39 were diagnosed by clinical examination alone. • 46 false-positive diagnoses based on a clinical examination. • Pulse-oximetry and echocardiography are accurate and cost-effective methods of diagnosing CHD including an ASD. • Early detection through newborn screening can improve the outcome. Treatment • Elective surgical repair is done in preschool years or when diagnosed in older adults.The latest studies have shown that it is better if left untreated until adulthood and repaired after manifestation of symptoms. • 2-4 hours to complete under GA. • Or percutaneous procedure that takes 30 mins. • 2 main types of ASD closure devices: Amplatzer® Septal Occluder System and the HELEXTM Septal Occluder. • Exclusion criteria: very large ASD, blood clots in the heart, other heart defects, if patient’s heart structure won’t allow the device (i.e not enough atrial septal tissue present), active infection anywhere in the body, bleeding disorder or the patient is not suitable for aspirin. • Also important is endocarditis prophylaxis. AGA Medical Corporation Childhood Vs adulthood • The best point in time to treat and aim the closure of the defect is controversial. • Some doctors support the theory that the sooner is treated the better the quality of life for the patient. • However, recent studies have shown that the outcome is better after manifestation of the symptoms. This theory is becoming more accepted since adulthood is a better time to evaluate the ASD in terms of its size, severity and impact on the patient. Complications of transcatheter occlusion Journal of American College of Cardiology. 2002. March 20,vol 39,No 6. 1061-5 • CardioSEAL/STARFlex (CS/SF) devices were used in 159 patients and Amplatzer Septal Occluder (ASO) in 258 patients. The study took place from December 1996 until January 2001. • Complications were: • Embolization in the main pulmonary artery(3.5%). • Malposition(3.5%). • Arrhythmias especially atrial fibrillation (in 6 patients). Continued.. • Pericardial effusion (in 2 patients). • Thrombus formation was seen in a 67-year old patient. To avoid this complication antiaggregation therapy was advised one day before the procedure. • Rupture of sizing balloon (in one patient). • Right iliac vein dissection, groin hematoma due to human mismanagement (in 3 patients). • One sudden death after 1.5 years. Complications if ASD left untreated • Enlargement of the right atrium and the right ventricle. • Right heart overload. The right side of the heart has to work harder to pump extra blood to the lungs, especially as resistance in the pulmonary artery increases. Over time, the heart may become overworked, and function may become impaired. • Arrythmias • Stroke • Pulmonary artery hypertension Migraines and ASD • In a study that took place in the University Hospital Gasthuisberg of Belgium, 2005,a migraine prevalence of 30% was found in patients with secundum type ASD. After percutaneous closure with Amplatzer ASD Occluder the prevalence remained the same but the frequency of migraine attacks were reduced significantly over a follow-up period of at least 2 years. Prognosis • Spontaneous closure may occur in the first year of life. • Most patients are asymptomatic until adulthood. • Symptoms may develop in the 3rd decade. • Pulmonary hypertension, atrial dysrhythmias, heart failure, and tricuspid and mitral regurgitation are all potential findings. • Post-operative results are excellent. The future: ASD and Genetics Nature Genetics. 2005. Feb. 37, 425-8. Mutation in myosin heavy chain 6 causes ASD. • Mutation on 14q12 was found in a family with dominantly inherited ASD. A missense mutation 1820N in α-myosin heavy chain (MYH 6) a structural protein that is highly expressed in the developing atria. • The factor that regulates MYH 6, called transcription factor TBX-5, was also found to play a role. • The data provide evidence for a connection between a structural protein, a transcription factor and ASD. There are still a lot of questions to be answered. Artial Septal Defect J.A. Moutiris, MD, MSc, PhD, FESC Department of Cardiology Paphos General Hospital