Congenital heart surgery has evolved into its own specialty requiring unique techniques and skills. Recognizing the need to establish a special certification in congenital heart surgery, the American Board of Thoracic Surgery began the process in 2005, eventually granting the first certifications to qualified applicants in 2009. The American Council for Graduate Medical Education and the Thoracic Surgery Residency Review Committee have now approved specific training programs throughout the United States that will help to ensure the proper training of congenital heart surgeons for the future.

A 3-year-old patient is described with an unusual form of co-arctation due to hypoplasia of the transverse arch and fibromuscular dysplasia involving a long segment of the thoracic aorta. Surgical repair required resection of the aorta from the distal transverse arch to the mid-descending thoracic aorta, and replacement with a 16-mm Dacron interposition graft. This case demonstrates the importance of preoperative evaluation of the entire aorta in the presence of co-arctation due to fibromuscular dysplasia.

The outlook for patients with hypoplastic left heart syndrome has dramatically improved over the past two decades. Universally fatal only 25 years ago, since that time outcomes for staged palliation have shown consistent improvement. Recent reports show that eight to nine patients from every ten can now leave the hospital after the Norwood procedure.1 Attrition following the Norwood procedure, nonetheless, remains significant, with from five to fifteen percent of patients dying between the first and second stages of the Norwood sequence.1–4 Only three-quarters of the patients undergoing surgery for hypoplastic left heart syndrome survive after five years, even at the centres reporting the best outcomes for the Norwood procedure.1,5 In addition to the deaths, some patients are unable to progress through the three stages of reconstruction, and may require cardiac transplantation, or have no options for further therapy. There are many causes for these mortalities and morbidities following the Norwood procedure, including elevated pulmonary vascular resistance, cardiac arrhythmias, coronary arterial insufficiency, right ventricular failure, right ventricular volume overload due to shunt-dependent physiology, and tricuspid valvar regurgitation. Many of these factors are interrelated, and may form feedback loops, which serve to propagate their adverse effects on patients with hypoplastic left heart syndrome.

The congenital cardiac malformation characterized by discordant connections between the atriums and ventricles, as well as those between the ventricles and the arterial trunks, has been given many names. The terms atrioventricular discordance, l-transposition of the great arteries, ventricular inversion, and congenitally corrected transposition have all been used. Regardless of terminology, this complex congenital anomaly has only recently been studied to analyze the long-term effects of its natural history and outcomes following traditional surgical repair of the associated malformations which serve to uncorrect the circulatory pathways. As more patients survive into adulthood, the effects of this condition are now better understood, and the surgical approaches used in the past are being re-examined in light of longer-term follow up.

the techniques of computational fluid dynamics are among the most powerful tools available to engineers dealing with the motion of fluids and the exchange of mass, momentum, and energy. they have recently been shown to have an increasing number of applications to the human cardiovascular system, including the fluid dynamics of surgical reconstruction of congenitally malformed parts of the cardiovascular system. in vitro models are the alternative laboratory tools with which to study fluid dynamics. the advantages of computational fluid dynamics over the in vitro models are the easy quantification of haemodynamic variables, such as rates of flow, pressure, and distribution of shear stress, and changes in geometric and fluid dynamics parameters. furthermore, using computational fluid dynamics allows the development of three-dimensional models to reproduce both the complex anatomy of the investigated region and the details of the surgical reconstruction, especially with the recent developments in magnetic resonance imaging. on the basis of the results, it is possible quantitatively to evaluate the surgical correction. this technology, which benefits greatly from the continuous improvement in hardware and software, enables cardiovascular experts and bioengineers to look at the fluid dynamics of various cardiovascular regions with increasing sophistication.

efforts to treat babies with hypoplastic left heart syndrome began over 30 years ago, when surgeons first reported limited success with palliative surgery. cayler et al. performed an anastomosis between the ascending aorta and the right pulmonary artery, bilateral banding of the pulmonary arteries, and an atrial septectomy. their follow-up extended for only 7 months, but the authors predicted that the rapid advances being made in cardiac surgery at the time heralded a new era where hypoplastic left heart syndrome would be cured. unfortunately, that prediction remains far from true today, although dramatic advances have been made in the therapy of this otherwise lethal condition.

the norwood procedure involves three separate stages of operative corrections. the first stage involves re-fashioning the pulmonary trunk into a neo-aorta so that it is possible to establish an unrestricted systemic circulation. an interpositional, or systemic-to-pulmonary arterial, shunt is then created between the neo-aorta and the pulmonary arteries to allow pulmonary perfusion and gas exchange. two of the available options for the systemic-to-pulmonary shunt are the central shunt and the right modified blalock-taussig shunt. in the setting of a central shunt, pulmonary perfusion is derived from a conduit placed between the pulmonary arterial bed and the neo-aorta whereas, in the modified blalock-taussig shunt, the conduit is interposed between one of the pulmonary arteries and the brachiocephalic artery. in subsequent stages, pulmonary perfusion is provided directly by deoxygenated blood. this is achieved by connecting, first, the superior caval vein, and then the inferior caval vein, to the pulmonary arteries. it is usually during the second stage that the systemic-to-pulmonary shunt is removed.