The secret of success of heart failure therapy: A lesson for ACHD?

Congenital heart disease Eminence-based medicine Evidence-based medicine Randomized trials Populations The eminent pioneersAround one in 100 babies is born with some form of congenital heart disease.As such congenital heart diseases are the most common birth defects.In the past, mortality was high and just a few children reached teenage years, or adulthood.Indeed, until the 1930s there was no remedy for any of the different forms of congenital heart disease.Congenital heart disease only began to become a treatable condition when on August 8th, 1938 the surgeon Robert Edward Gross for the first time successfully ligated a persistent Ductus Arteriosis (Fig. 1) [1].This was a breakthrough; indeed, traditional surgeons such as Theodor Billroth (1829-1894) used to say "Any surgeon who tries to sew a wound in the heart, will lose the respect of his colleagues for good".After this seminal first surgical procedure, Clarence Crafoord and G. Nylin at the Karolinska in Stockholm corrected a patient with coarctation of the aorta in 1944.In the same year, Helen Taussig, who established pediatric cardiology at Johns Hopkins University in Baltimore developed with the surgeon Alfred Blalock the so-called Blalock-Taussig shunt which allowed for palliation of tricuspid atresia [2].After the initial successes with these extracardiac operations, surgery of the atrial septum defect was the next frontier.After several attempts with closed techniques, F. John Lewis for the first time openly closed an atrial septal defect in a 5-year-old girl on September 2, 1952 under hypothermia with closed flow (Fig. 1) [3].However, to surgically correct more complex congenital lesions, novel medical technologies were required, in particular cardiac cathe-terization and imaging, Werner Forssmann performed the first right heart catheterization on himself in 1923 [4]; thereafter the procedure was developed for clinical use by Andr e F. Cournand and Dickinson W. Richards.Coronary angiography was then introduced by Mason Soanes in 1953 and cardiology was ready to provide the structural and hemodynamic information for bypass and congenital cardiac surgery [5].In the meantime, also in the 1950s, balloon dilatation of the pulmonary valve was first performed, as was balloon atrial septostomy a few years later, to promote the mixing of the blood at the atrial level, to improve outcomes of transpositions of the great arteries [6].The real breakthrough for congenital heart surgery, however, came through the development of the first heart and lung machines by John Gibbon in the United States [7] and Ake Senning and Clarence Crawford in Stockholm in the 1950s (Fig. 1).This allowed initially for palliative and, later, corrective surgery of more complex congenital heart lesion.The successes of these innovative surgical pioneers is truly impressive and has been primarily been achieved in collaboration with ingenious paediatric cardiologists, but mainly through eminence-based medicine.Indeed, clinical intuition and hemodynamic reasoning were mostly the basis of their decisions.Rarely, if ever, have trials according to today's standards comparing different approaches or evaluating indications been performed in this patient population.However, the fact that a century ago less than 5% of children with congenital heart disease reached adulthood, and now this is possible for the overwhelming majority of such patients, speaks for itself.