Cervical aortic arch is a rare congenital anomaly. We present a case of left-sided cervical aortic arch, found in a patient with mitral regurgitation, mitral stenosis, and a regurgitant bicuspid aortic valve. There was atypical obstruction proximally within the arch. The obstructive segment was resected, and corrected by performing an end-to-side anostomosis. Mitral valvoplasty was performed in the same surgical procedure. To the best of our knowledge, a cervical arch has not previously been described with such atypical obstruction, and in association with multiple lesions involving the left heart.

An acute injury to a coronary artery was recognized during the surgical construction of the Fontan circulation. Surgical manipulation of the site of injury was not successful in restoring normal myocardial blood flow. A stent was therefore placed intraoperatively under direct vision, with restoration of normal coronary arterial flow acutely and at short-term follow-up.

Aortic coarctation is a recognized arteriopathy in patients with Williams’ syndrome. We present an adolescent with Williams’ syndrome who developed rapid restenosis after primary stenting of coarctation of the aorta. We believe such restenosis within the stent is due to a proliferative response of the abnormal aortic wall. Attention should be paid to the potential for restenosis during follow-up after stenting of aortic coarctation in patients with Williams’ syndrome.

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.

Hypoplastic left heart syndrome is the term introduced by Noonan and Nadas1 to describe a spectrum of cardiac anomalies characterized by varying degrees of significant underdevelopment of the left heart and aortic arch. These cardiac anomalies include mitral valvar disease, left ventricular hypoplasia, aortic stenosis at subvalvar, valvar, and supravalvar levels, hypoplasia of the ascending aorta and aortic arch, and aortic coarctation. Although descriptive, hypoplastic left heart syndrome suffers from being a very unspecific term, since it encompasses multiple degrees and combinations of abnormalities involving the left sided structures. In an attempt to provide gradings of severity, Kirklin and Barratt-Boyes2 categorized these patients falling into the syndrome into four classes, according to whether obstruction was found at one, two, or more levels, or whether there is aortic atresia. Then, in 1998, Tchervenkov and colleagues3 introduced the term hypoplastic left heart complex to describe a set of patients falling within the spectrum of hypoplasia of the left heart, but in the absence of intrinsic aortic or mitral valvar stenosis, this concept subsequently being endorsed by the International Committee established by the Society of Thoracic Surgeons, together with the European Association of Cardiothoracic Surgery, to rationalize the approach to nomenclature and databases.4 The analysis offered by Tchervenkov et al.3 showed that, in certain circumstances, there are potentially patients considered to have hypoplasia of the left heart who might be candidates for biventricular repair. If this is the case, then it is important to establish how this subset can be recognized, and how they are best treated.

I am grateful for the opportunity to participate in this debate forum, and for the honor of exchanging concepts with Len Bailey. This debate, fortunately, represents a “win-win” situation. The innovations and evolution of both replacement of the heart and reconstructive surgery for hypoplastic left heart syndrome are stories with lots of winners. The entire field of transplantation biology, the entire scope of management of the newborn with complex congenital heart disease, including both medical and supportive therapy and surgical strategies, has each been advanced tremendously as a consequence of the introduction and refinement of these two very different therapies for hypoplastic left heart syndrome. So, it is a debate that cannot be lost.

We present the eighth published case of divided left atrium co-existing with tetralogy of Fallot. This is the first report of preoperative echocardiographic diagnosis of this unusual combination of defects. Demonstration of a partition within the left atrium is imperative for successful repair of the combined lesions. We draw attention to the need for careful echocardiography in patients where an obstruction to pulmonary venous drainage would dramatically affect the outcome subsequent to surgical correction.

We report a patient in whom the circumflex coronary artery arose anomalously from the right aortic sinus. The patient also had atrial and ventricular septal defects in the setting of Eisenmenger physiology. We recommended transplantation of the heart and lungs.

the hypoplastic left heart syndrome includes a spectrum of underdevelopment of the left-sided cardiac structures, characterized by aortic valvar atresia or severe stenosis with a small ascending aorta and hypoplasia of the left ventricle. without surgical treatment, practically all infants with this complex cardiac anomaly die within the first month of life. since norwood et al. reported successful surgical palliation in 1981, multi-staged reconstructive surgery, involving the norwood procedure as the first stage, followed by a bi-directional glenn anastomosis and subsequent completion of the fontan circulation, has become the established surgical treatment.

A 14-year-old female patient underwent surgical treatment of multiple atrial septal defects associated with unroofed coronary sinus and pulmonary valvar stenosis. One of the defects was that of the superior oval fossa and the other a large ellipsoidal defect positioned inferior to the inferior rim of the oval fossa. The patient underwent primary closure of the defects with a favorable result. To the best of our knowledge, this is the first surgical experience of an unusual atrial septal defect or the vestibular defect.

We report two more patients with the association of postaxial polydactyly and atrioventricular septal defects. Additional cardiac findings, not previously described, were tetralogy of Fallot in the first case, and double orifice right atrioventricular valve in the second child.

Thank you for asking me to make the case for cardiac transplantation in this debate. Marshall, that was a truly comprehensive and spectacular defense of the value of reconstructive surgery in babies born with hypoplastic left heart syndrome. It is clear that the potential for babies born with this complex and lethal malformation has improved dramatically over the past 2 decades. The improvement in outcome is a direct result of complementary surgical strategies. Marshall Jacobs has constructed a stellar review outlining the important issues relating to the ever-evolving staged palliative reconstruction for hypoplastic left heart syndrome. And, he appealed to those present at the Symposium with some images of extraordinary children who represent the most favorable outcomes seen today after staged reconstructive surgery. It would be inappropriate for me to criticize or condemn any feature of these favorable outcomes.

As far as we are aware, a bicuspid aortic valve has not previously been reported in the setting of tetralogy of Fallot and pulmonary atresia. We describe this association in a newborn who presented with a murmur and cyanosis. Echocardiography showed tetralogy of Fallot with pulmonary atresia, and also a moderately stenotic bicuspid aortic valve. The patient underwent open-heart surgery guided by transesophageal echocardiography. Postoperatively, there was only mild obstruction across both outflow tracts. We have also reviewed the pertinent data from our Jesse E. Edwards Registry of Cardiovascular Disease to establish the incidence of bicuspid aortic valve in the setting of obstruction of the right ventricular outflow tract, finding the association in no patients with pulmonary atresia and tetralogy of Fallot, in 0.7% of those with tetralogy of Fallot and pulmonary stenosis, but in 6.6% of those with pulmonary atresia and intact ventricular septum.