Air embolism secondary to mechanical ventilation is a rare but well-described complication in premature infants. We describe the echocardiographic appearance of venous air embolism manifesting as acute obstruction of the right ventricular outflow tract in such a premature infant, and review the pathophysiology of acute obstruction of the right ventricular outflow tract secondary to the “air lock” phenomenon. Awareness of the pathophysiology and echocardiographic appearance of venous air embolism may aid in prompt recognition and potential therapy for this lethal complication of mechanical ventilation.

in the 17th century, william harvey discovered the circulation of the blood, describing at this time the pumping function of the heart and the “in series” disposition of the pulmonary and systemic circulations. these concepts provided the foundation for the development of modern cardiac physiology. during the three centuries that followed the discovery of circulation, many scientists studied and expanded our knowledge of cardiac and pulmonary function. with the description of complex congenital cardiac diseases, in particular functionally univentricular hearts, and with the development of their palliative surgical treatment, we have uncovered another type of cardiac physiology. in these cases, the circulation of the blood is characterized by an “in parallel” disposition of the pulmonary and systemic circulations, with direct venous–arterial connections in the absence of one pumping ventricle.

As I had hoped and anticipated, the previous contributions have shown that Len and I are involved in a debate, but not an argument. As I am asked in this rebuttal to respond to some of the very important points that Len has made, I shall do so. I think that he was very accurate in distilling, from the study of outcomes over 15 years in Philadelphia, the message that late attrition was quite low. Of the first cohort in that study, undergoing surgery way back in 1984, less than one-third survived. As those of you who are familiar with the study now, it has improved steadily, so that now at least four-fifths of those undergoing first stage palliations survive. But what is really important is that those children who got out to 5 years usually got out to 10. And nearly all of those that got out to 10 years were alive at 15 years. I have already pointed to evidence that rates of survival with respect to initial surgical palliation are improving steadily at centers throughout the United States of America, and also around the world. So that, were it not for the shortage of donor organs, we could get together again in a decade or two from now and debate the merits of these two forms of therapy based on a comparison of the late morbidities associated with the creation of the Fontan circulation, such as sinus nodal dysfunction, protein losing enteropathy, thromboembolic phenomenons, and so on, to those events occurring at later stages associated with organ replacement, such as graft atherosclerosis, lymphoproliferative disorders, late rejection, and so on.

We describe the successful management of two patients with infected modified Blalock-Taussig shunts by replacement of the graft combined with antibiotic therapy.

We describe an infant with an anomalous left coronary artery arising from the pulmonary trunk leading to myocardial ischaemia and development of an apical aneurysm of the left ventricle.

Clinical presentation in general is based on myocardial hypoperfusion resulting in ischaemia and infarction. When presenting in infancy, however, then the features, as in our patient, may be tachypnea, dyspnea, failure to thrive and irritability, especially during feeding. Then, again as in our patient, it is possible to miss the definitive diagnosis, which is made by echocardiography and cardiac catheterization. Reimplantation of the anomalous left coronary artery into the aorta usually results in improvement of left ventricular function over time. In our patient, the myocardial infarction was complicated by formation of an aneurysm, and reimplantation alone was not sufficient to restore cardiac function. Resection of the aneurysm greatly improved the hemodynamics.

The portrayal of a beautiful youngster performing uninhibited acrobatics based on Fontan physiology, as presented by Marshall Jacobs, is a brilliant and beautiful thing for us to see. It is, perhaps, all about will over physiology. But it is, nevertheless, happening for that child. Marshall mentioned the need for re-transplantation, whether the beginning strategy was transplantation or reconstructive surgery. Indeed, a relatively small percentage of children transplanted will require re-transplantation because of severe graft coronary disease. Remarkably, in the Loma Linda experience, 10-year actuarial survival for 26 patients following elective re-transplantation is over 85%, exceeding overall actuarial survival at 10 years for children following primary transplantation. Many of the transplanted infants, however, seem to be realistically hopeful that one heart will last their entire lifetime. Of course, the hope is that their's will be a long and healthy lifetime. The requirement for late transplantation following Fontan procedures, however, seems almost inevitable. We'll simply have to keep these children with Fontan circulation under surveillance to see when, in the course of their lives, transplantation will become necessary. Unfortunately, operative and long-term survival among children who are transplanted for failing Fontan physiology have, as yet, been somewhat suboptimal.

A young man had anomalous connection of the veins draining the upper lobe of the left lung to both a left-sided vertical vein and the left atrium. The ratio of pulmonary to systemic flows was 1.7 : 1, and he was symptomatic with evidence of volume overload of the right heart. He was successfully treated by percutaneous placement of an Amplatzer ductal occlusion device into the vertical vein.