Congenital: Cardiomyopathy
Commentary: Building evidence to support empiric observations—Molecular cross talk, or simply crossed wires?
Surgeons are reductionists; we know what works for patients, often on the basis of personal experience. We can sometimes suggest a plausible mechanism, citing basic mechanisms at work. Surgeons are also sceptics; we value clarity and simplicity and are quick to note when the “science” fails to demonstrate mechanisms supporting observations made in the clinical domain. There is a lot to be sceptical about, because so many procedures lack an evidence base or a defined mechanism of action.Commentary: A bandage for dilated left ventricular cardiomyopathy
In this month's edition of The Journal of Thoracic and Cardiovascular Surgery, Yerebakan and colleagues1 present their work studying the effects of central pulmonary artery banding on doxorubicin-induced left ventricular (LV) cardiomyopathy in juvenile animals. Their group used selective left main intracoronary injections of doxorubicin in 4-month-old sheep to induce LV dilation and impairment and then subjected the animals to either central pulmonary artery banding (CPAB) or a sham operation. Echocardiography conducted before the animals were killed and the hearts explanted demonstrated that CPAB improved the LV function and reduced the LV dimensions without an effect on the pressure-volume loop assessments.Surgical relief of left ventricular outflow obstruction in pediatric hypertrophic cardiomyopathy: The need for a tailored approach
Hypertrophic cardiomyopathy (HCM) is extremely heterogeneous with regard to both genetic origins and phenotypic expressions.1,2 The age of onset of the disease has been recently identified as a major determinant of prognosis, phenotype, and association with syndromic features. This has led to the recognition of pediatric HCM as a specific disease entity1 requiring specific management. Left ventricular outflow tract (LVOT) obstruction is a defining feature,3 which when severe requires surgical myectomy usually through a transaortic approach.Modified Konno instead of myectomy: Another tool in the box?
The modified Konno procedure to treat obstructive hypertrophic cardiomyopathy (HCM) has not been widely applied because the early and late results of extended septal myectomy have been excellent. In general, extended septal myectomy has been the criterion standard for patients with symptoms of all ages, including children with HCM refractory to medical therapy. With that said, the surgical management of HCM in children continues to be a challenge, particularly for those with Noonan syndrome. Laredo and colleagues1 are to be congratulated on their results, published in this issue of the Journal, of a series of 79 children who underwent a modified Konno procedure as a strategy to treat obstructive HCM.From genetics to prognosis
Cardiomyopathies in childhood are challenging cardiac disorders that carry high mortalities. Cardiac transplantation is the treatment of choice in case of intractable cardiac failure; however, the chronic shortage of donor organs hinders its unlimited application. Medical and surgical management strategies evolve steadily to improve the outcomes of our patients. Better risk stratification and novel alternatives for medical and surgical treatment options are therefore required.1,2
