Hypoplastic Left Heart Syndrome:
Refers to constellation of cardiac anomalies
–Marked hypoplasia or absence of LV and severe hypoplasia of ascending aorta/aortic arch
–Systemic circulation is perfused by RV through PDA
–Mixing of systemic and pulmonary venous blood in the RA
Term was introduced by Noonan and Nadas in 1958
Occurs in 7-9% of neonates diagnosed with heart disease
Without surgery HLHS is fatal
–Accounts for 25% of cardiac deaths in the first week of life
Most are diagnosed due to tachypnea and cyanosis in the 1st 24 to 48 hours after birth
Diagnosed by echocardiogram
Once PDA closes
–Diminished systemic perfusion
Demise if untreated
–Maintain ductal patency, PGE
–Maintain Oxygen saturation (75-85%)
Decreased pulmonary blood flow
May need sub-atmospheric oxygen (Nitrogen vs. CO2)
Avoid mechanical ventilation if possible
–Augment ascending aorta
–Amalgamate aorta and pulmonary artery
–Enlarge atrial communication
–Provide reliable source of pulmonary blood flow
RV to PA shunt (Sano Shunt)
The perioperative care, surgical techniques and technology in repair of complex congenital cardiac defects in neonates and infants have evolved considerably over the past 40 years
These changes have allowed us to repair many defects which were considered lethal even in the 1980’s. As the mortality rate is decreasing for treatment of these complex congenital cardiac defects, surgeons are challenged to improve not only the survival of their patients, but also their quality of life.
About Dr. Kamal K. Pourmoghadam
Kamal Pourmoghadam, MD, is a pediatric cardiac surgeon at The Heart Center at Arnold Palmer Hospital for Children. He is board certified in general surgery, cardiothoracic surgery and congenital cardiac surgery.
Dr. Pourmoghadam earned his bachelor’s degree from University of California, Berkeley, and his medical degree from Albany Medical College in New York. He trained for adult cardiac surgery at the University of Miami, Jackson Memorial Hospital in Miami, and for congenital cardiac surgery at the University of Washington, Seattle Children’s Hospital in Seattle.
Dr. Pourmoghadam is a professor of surgery at the University of Central Florida College of Medicine, practicing congenital cardiac surgery for over twenty years and has been active in clinical research. He has extensive experience in neonatal and infant cardiac surgery and has special interest in the repair of single ventricle physiology patients and research in univentricular hearts.
News report about Dr. Pourmoghadam: http://www.tiogapublishing.com/features/the_marketplace/covington-tot-returns-home-to-pennsylvania-after-lengthy-oklahoma-hospital/article_04865c00-0ae5-11e1-aec8-001cc4c002e0.html