Claudia Stöllberger

Serious Complications from Dislocation of a Watchman Left Atrial Appendage Occluder

Left atrial appendage (LAA) elimination is regarded to protect patients with atrial fibrillation (AF) from stroke or embolism. The Watchman occluder is a device for percutaneous LAA occlusion and is at present investigated in the PROTECT AF trial. In a 78‐year‐old man, embolization of the Watchman device occurred 10 minutes after implantation. At emergency cardiac surgery, the device was removed from the aortic valve and an aortic bioprosthesis and a pacemaker had to be implanted. One year postoperatively, he still suffers from heart failure. This case shows that percutaneous LAA occlusion may result in serious complications.

Quantitative electrocardiographic measures, neuromuscular disorders, and survival in left ventricular hypertrabeculation/noncompaction.

Left ventricular hypertrabeculation/noncompaction (LVHT) is frequently associated with neuromuscular disorders (NMDs) and electrocardiographic (ECG) abnormalities. Quantitative ECG‐measures (QEMs) are risk markers for mortality in cardiomyopathies. We measured QEMs in the ECGs in LVHT patients with and without NMDs.

Evolution of Electrocardiographic Abnormalities in Association with Neuromuscular Disorders and Survival in Left Ventricular Hypertrabeculation/Noncompaction

Left ventricular hypertrabeculation/noncompaction (LVHT) is frequently associated with neuromuscular disorders (NMDs) and electrocardiographic (ECG) abnormalities. The prognostic relevance of newly developed ECG abnormalities in LVHT and its dependency on NMD is largely unknown. Aim of the following retrospective cohort study in LVHT patients was thus to assess the development of new ECG abnormalities and its dependency on NMD and survival

Wearable Cardioverter-Defibrillator in a Patient with Left Ventricular Noncompaction/Hypertrabeculation, Coronary Artery Disease, and Polyneuropathy

A 55‐year‐old Caucasian male with coronary heart disease was admitted because of dyspnea for 4 weeks. Echocardiography showed a dilated left ventricle with an ejection fraction of 34% and apical left ventricular hypertrabeculation/noncompaction with an apical thrombus. Neurologic examination revealed positional tremor and generally reduced tendon reflexes. During 8 weeks, his condition improved under pharmacotherapy. The patient was skeptical about implantable cardioverter‐defibrillator (ICD) and expected further improvement from pharmacotherapy. Thus, he received a wearable cardioverter‐defibrillator (WCD). We conclude that a WCD might be useful in noncompaction patients in whom improvement of systolic dysfunction is expected or who are skeptical about ICDs.

Repeated radiofrequency ablation of atrial tachycardia in restrictive cardiomyopathy secondary to myofibrillar myopathy.

Myofibrillar myopathy is characterized by nonhyaline and hyaline lesions due to mutations in nuclear genes encoding for extra‐myofibrillar or myofibrillar proteins. Cardiac involvement in myofibrillar myopathy may be phenotypically expressed as dilated, hypertrophic, or restrictive cardiomyopathy. Radiofrequency ablation of atrial fibrillation and flutter has so far not been reported in myofibrillar myopathy. We report the case of a young female with myofibrillar myopathy and deteriorating heart failure due to restrictive cardiomyopathy and recurrent atrial fibrillation and atrial tachycardias intolerant to pharmacotherapy. Cardiac arrhythmias were successfully treated with repeat radiofrequency ablations and resulted in regression of heart failure, thus postponing the necessity for cardiac transplantation.

Does the Pathogenesis of Atrial Fibrillation Vary Between Hereditary and Non-Hereditary Cardiac Disease?

With interest we read the article by Manugeurra et al. about inherited structural heart disease (ISHD) with potential atrial fibrillation (AF) occurrence.1 Reading this overview about the association of AF with hypertrophic cardiomyopathy, dilated cardiomyopathy, arrhythmogenic right ventricular cardiomyopathy, Fabry disease, hemochromatosis, amyloidosis, left ventricular hypertrabeculation/noncompaction (LVHT), Holt–Oram syndrome, mitochondrial disease and tetralogy of Fallot, the following questions arise: In view of the worse prognosis of AF in patients with ISHD compared with sinus rhythm, are there any data indicating which preventive measures can be taken to prevent development of AF? Should we screen families of patients with ISHD for clinically silent cardiomyopathy and follow them for the development of AF? Are there any data if rhythm control by pharmacologic intervention or by electrical cardioversion is beneficial for these patients?2 Is catheter ablation of AF a therapy which we should recommend to patients with ISHD?3 Are there any studies comparing electrophysiological findings of patients with ISHD with patients with AF due to other cardiac disorders? AF is associated with the risk for stroke or embolism. Are there any data if patients with ISHD are more at risk for thromboembolism than patients with AF due to other etiologies? Is it appropriate to apply embolic risk scores in all patients with ISHD, as has been shown to be the case in patients with LVHT?4 According to current guidelines, however, patients with hypertrophic cardiomyopathy and AF should receive anticoagulant therapy irrespective of their CHADS2and CHA2DS2VASc scores.5 As pointed out by the authors, ISHD may be confined to the heart or manifest as cardiac involvement of systemic disorders. Which investigations are recommended to assess the etiology of the different phenotypes of cardiomyopathies?

Familial Himalayan P Wave and Left Ventricular Hypertrabeculation/Noncompaction

“Himalayan P waves,” are reported in congenital heart disease and cardiomyopathies.

Is Surgical Removal of the Noncompacted Layer the Clue to Treat Left Ventricular Hypertrabeculation/Noncompaction?

Dear Editor, With interest we read the article by Gan et al. about surgical restoration as possible treatment for left ventricular hypertrabeculation/noncompaction (LVHT). We have the following questions: It would be interesting to know if progressive exercise intolerance was attributable only to cardiac dysfunction or any extracardiac disease, in particular a neuromuscular disorder with which LVHT is frequently associated, if the patient was investigated neurologically and if cardiac or muscle disease was present in any of the relatives. Information should be given, if heart failure developed suddenly or symptoms progressed slowly, and if echocardiographic recordings were available to assess if LVHT was congenital or acquired. Did the electrocardiogram show any abnormalities? Since it remains unclear if mitral regurgitation was due to a valvular or ventricular problem, we would like to know inmore detail themorphology of themitral valves, the chordae tendineae, and the papillary muscles, especially if LVHT also affected the papillary muscles. Speckle-tracking is a new technique with inherent limitations. Was it decided to surgically remove LVHT preoperatively or intraoperatively, based only on the reduced early diastolic strain-rate or were other indicators for diastolic dysfunction considered, like Doppler sonography or cardiac catheterization? It should be clarified whether diastolic dysfunction was due to impaired relaxation or restriction. There is a discrepancy between the echocardiographic image showing trabeculations protruding into the left ventricular cavity and the diagram showing horizontal trabeculations crossing the left ventricular cavity. Were all segments of noncompacted myocardium removed or did some segments remain? The histological appearance of the resected trabeculations should be described. Were the trabeculations associated with subendocardial fibrosis, a frequent finding in LVHT? The postoperative pharmacotherapy of the patient, including anticoagulation therapy, would be interesting to know. The authors mention another case of LVHT in whom the left ventricular cavity was partially excluded. Both cases, so far reported, recovered well after surgery. It remains unclear, however, whether in the present case clinical improvement was due to correction of the mitral regurgitation or due to the resection of LVHT. Since the etiology and pathogenesis of LVHT is largely unknown we do not know if the trabecular meshwork is a compensatory, adaptive attempt of the failing myocardium or the reason for heart failure. In view of the limited therapeutic options for LVHT patients with severe heart failure and disadvantages of cardiac transplantation due to shortage of donor organs and necessity for lifelong immunosuppression, we consider further investigations about LVHT resection useful, preferentially starting in genetically engineered mouse models.

Left Atrial Appendage Occlusion by the WATCHMAN Device: A Beneficial Procedure?

(1) What was the indication for LAA closure because none of the patients had contraindications for warfarin therapy? Were the patients included in the PROTECT-AF trial?2 (2) How was incomplete LAA closure defined? Was demonstration of a gap by 2D echocardiography sufficient or was in addition the presence of a color Doppler jet around the device required? In relation to the LAA dimension, we doubt that a jet width ≤5 mm is always irrelevant. Thus, in how many patients, the LAA was completely sealed at the time of implantation and how many patients developed gaps and/ or color jets around the device during follow-up? (3) The rationale for the combined therapy with warfarin and aspirin for 45 days after device implantation remains unexplained. Possibly this antithrombotic combination prevents complete LAA sealing. In addition, it remains unclear, how long the combination of aspirin and clopidogrel was given thereafter. This dual antiplatelet therapy is no more protective against stroke and embolism than warfarin alone but associated with more bleeding events.3 So it would be interesting to know how many patients in the present study developed bleeding events. (4) What was the indication for performing TEE in the patient showing a thrombus on the LAA device 21 months after implantation? Which antithrombotic therapy did this patient receive at the time of the TEE study? Overall, it would be interesting to know in how many cases