ABSTRACT
INTRODUCTION: This study evaluates the accuracy of iECGs in comparison to the gold standard ECG in adult patients with congenital heart disease and recommends the appropriate iECG derivation based on the patient's characteristics. METHODS: In 106 adults (51 female, 55 male) with congenital heart disease, a gold standard 12-lead ECG was recorded, followed by three iECGs with the Apple Watch series 4, which correspond to Einthoven leads I, II, and III. Two experienced and independent cardiologists analyzed the time intervals, amplitudes, and polarities of the ECG parameters as well as the rhythm type and correlated the patient characteristics with the iECG parameters. RESULTS: The iECG parameters of all three iECG leads correlate strongly with those of the gold standard ECG, with exception of the P and T wave durations. We demonstrated that the informative value of the individual iECGs was independent of the patient's characteristics, in particular the heart axis, anatomy, and situs, even if the quality of the Einthoven III-like derivation was partially inadequate. The automatic rhythm analysis of the Apple Watch and the heart rhythm classification of a standard ECG analyzed manually by a cardiologist corresponded in 77%. CONCLUSION: iECG recordings of adults with congenital heart disease provide comparable results with Einthoven recordings I, II, and III of the 12-lead ECG and current data encourage the use of the Apple Watch not only in patients with structurally normal hearts but also in patients with congenital heart disease.
ABSTRACT
The Marfan syndrome is an inherited disorder of the connective tissue which is mainly caused by a mutation in the fibrillin-1 gene. The defect in the connective tissue protein can lead to several organ dysfunctions. For the life expectancy, the cardiovascular aspect is of paramount importance. Patients with Marfan syndrome may develop aortic aneurysms and valvular heart defects. The risk of aortic aneurysms consists in the development of aortic dissection or rupture with their fatal consequences. Besides the cardiovascular manifestation, the skeletal and ocular system can also be affected. The skeletal manifestation is often characterised by long limbs, arachnodactyly, and abnormal joint flexibility along with other signs. Patients may also have dislocated lenses, ectasia of the dural sac, stretch marks, spontaneous pneumothorax, recurrent hernia, or a family history suspicious for Marfan. During the past years, other related connective tissue disorders with analogous organ manifestation have been described (e.g., Loeys-Dietz syndrome). In this article we present the basic knowledge about these connective tissue disorders, and we mention new insights in the recently explored pathophysiology of the disorder which is a possible target for future medical treatment options. Furthermore, recent new concepts for the prophylactic treatment of the aortic manifestation are explained.
