A rare case of cardiac fibroma diagnosis in Gorlin-Goltz syndrome with information on management.

Gorlin-Goltz syndrome is a rare autosomal dominant disease characterized by odontogenic keratocysts and basal cell carcinoma as well as ophthalmic and neurological implications. The following article presents the case of a 20-year-old female with Gorlin-Goltz syndrome incidentally found to have a cardiac mass. An ECG showed diffuse T-wave inversions in the lateral leads despite a lack of any acute coronary symptoms in the patient. Echocardiogram, cardiac MRI and CT scan confirmed a nonvascularized, smoothly marginated mass arising from the left ventricular apex without any hemodynamic compromise. A whole-body PET scan further demonstrated localized hyperactivity associated with a cardiac fibroma without any evidence of metastasis. The cardiac fibroma was surgically excised for definitive management to prevent the possibility of sudden cardiac death and congestive heart failure.

Cardiac fibroma presents as a benign tumor of the heart. Although tumors of the heart are uncommon, patients who have Gorlin­Goltz syndrome are at a higher risk of developing these fibromas. In the following article, the authors discuss the presence of this rare cardiac fibroma in a 20-year-old female patient with Gorlin­Goltz syndrome. The patient's tumor was found incidentally during a CT scan after she presented with symptoms of flank pain. A follow-up was later conducted with cardiology and cardiac surgery. Several tests were performed to scan the exact tumor location in the heart. Finally, the tumor was removed, and the patient recovered after surgery. Later, the patient was diagnosed with depression and needed medicine to cope with emotional trauma from multiple surgeries.

Is there still a role for renal artery stenting in the management of renovascular hypertension - A single-center experience and where do we stand?

BACKGROUND: Renal artery (RA) stenosis has been implicated in the pathophysiological mechanism for resistant hypertension. Despite the increasingly diagnosed frequency of hemodynamically significant lesions, the value of RA revascularization remains controversial. Our group had previously demonstrated significant blood pressure (BP) reduction in a retrospective cohort of appropriately selected patients undergoing RA stenting up to 18-months of follow-up. We herein present long-term clinical outcomes data 5-years post revascularization on 26 subjects who continued follow-up at our institution. METHODS: Retrospective analysis was performed on subjects who underwent RA stenting at our institution for hemodynamically significant (≥70%) RA stenosis and systolic hypertension on ≥3 antihypertensive agents. Clinical outcome data for systolic blood pressure (SBP), diastolic blood pressure (DBP), creatinine level and number of antihypertensive drugs was assessed prior to and then later at 6-12 months and 3-5 years post RA stenting. RESULTS: Mean age was 69 ±â€¯9 years; 27% (7/26) were male. Median follow-up was 5.1 years. Blood pressure reduction was sustained at long-term follow-up (135/70 ±â€¯18/11 mmHg) compared to initial reduction noted at 6-months (136/69 ±â€¯16/8 mmHg; p ≤0.01 for both) and from baseline (162/80 ±â€¯24/18 mmHg; p ≤0.001 for both). The number of antihypertensive agents also decreased from 4.1 ±â€¯1.0 to 2.7 ±â€¯2.1 (p = 0.002) at 6-months and was sustained at long-term follow-up, 3.4 ±â€¯1.2 (p = 0.03) with no difference in renal function between short- and long-term follow-up compared to baseline. CONCLUSIONS: This study shows sustained benefit of RA stenting in BP reduction in an appropriately selected cohort with significant stenosis ≥70% and uncontrolled hypertension on multiple medications on long-term follow-up.

Is symptom to balloon time a better predictor of outcomes in acute ST-segment elevation myocardial infarction than door to balloon time?

INTRODUCTION: Current guidelines for the treatment of ST-segment elevation myocardial infarction (STEMI) recommend early revascularization with a door-to-balloon (D2B) time of 90 minutes or less in patients undergoing primary percutaneous coronary intervention (PPCI). The focus of most studies has been D2B time. Because of the large variability in the time between symptom onset and presentation, we sought to determine the effect of symptom-to-balloon (S2B) time on presentation and outcomes as a potentially more clinically relevant parameter. METHODS: We conducted a retrospective study of 106 patients who were diagnosed with an acute STEMI, had a documented S2B time and who underwent a PPCI at a tertiary hospital from the period of January 2014 to December 2014. S2B time was defined as the time interval beginning from the episode of chest pain that led the patient to present to the emergency department to the time of the first balloon inflation. We categorized our patients into 2 main groups depending on whether S2B time was greater or less than 240 minutes. They were further subdivided into 2 groups depending on the site of the culprit lesion (left anterior descending LAD vs. non-LAD). RESULTS: There was no difference between the two main groups in regard to the left ventricular ejection fraction (EF) on presentation, length of stay, and readmission with heart failure or chest pain. However, when S2B time was greater than 240 min, there was a statistically significant difference in left ventricular ejection fraction (EF) between LAD and non-LAD stenosis with a mean of 38.4% and 49.3% respectively (P=0.01). No relationship was found between S2B time and gender or age. CONCLUSION: Although D2B time is a well-established clinical parameter, S2B time may be expected to be a more accurate predictor of outcomes. However, in our study, S2B time of >240 minutes only predicted a significant worse EF (and presumably mortality) when the culprit vessel was the LAD. Further studies are needed to better elucidate the relation of S2B time to clinical outcomes.

Validating Left Ventricular Filling Pressure Measurements in Patients with Congestive Heart Failure: CardioMEMS™ Pulmonary Arterial Diastolic Pressure versus Left Atrial Pressure Measurement by Transthoracic Echocardiography.

BACKGROUND: Routine ambulatory echocardiographic estimates of left ventricular (LV) filling pressures are not cost-effective and are occasionally fraught with anatomic, physiologic as well as logistical limitations. The use of implantable hemodynamic devices such as CardioMEMS Heart Failure (HF) System has been shown to reduce HF-related readmission rates by remote monitoring of LV filling pressures. Little is known about the correlation between CardioMEMS and echocardiography-derived estimates of central hemodynamics. METHODS: We performed a prospective, single-center study enrolling seventeen participants with New York Heart Association functional class II-III HF and preimplanted CardioMEMS sensor. Simultaneous CardioMEMS readings and a limited echocardiogram were performed at individual clinic visits. Estimated left atrial pressure (LAP) by echocardiogram was calculated by the Nagueh formula. Linear regression was used as a measure of agreement. Variability between methods was evaluated by Bland-Altman analysis. RESULTS: Mean age was 74 ± 9 years; 59% (10/17) were males. LV systolic dysfunction was present in 76% (13/17) of subjects. Mean PAdP was 18 ± 4 mmHg and 19 ± 5 mmHg for CardioMEMS and echocardiographic-derived estimates, respectively, with a significant correlation between both methods (r2=0.798, p ≤ 0.001). CONCLUSIONS: Our study illustrates a direct linear correlation between PAdP measured by CardioMEMS and simultaneous measurement of LV filling pressures derived by echocardiography.

Effect of sacubitril/valsartan on cardiac filling pressures in patients with left ventricular systolic dysfunction.

BACKGROUND: Sacubitril/valsartan is the newest neurohormonal agent approved for therapy in patients with heart failure with reduced ejection fraction (HFrEF). Little is known about its acute and incremental hemodynamic effects. We aimed to evaluate the change in hemodynamic profiles measured using an implanted monitoring device in HFrEF patients initiated on sacubitril/valsartan therapy. METHODS: We prospectively enrolled 13 subjects with HFrEF and pre-implanted CardioMEMS™ device on maximally tolerated guideline-directed medical therapy and no contraindications to sacubitril/valsartan therapy. Transmitted pulmonary artery diastolic pressures (PAdP) from CardioMEMS™ were averaged and compared for one week before and after initiation of sacubitril/valsartan, as well as after change in medication strength and finally at three months. RESULTS: Sacubitril/valsartan dose increase was tolerated in 7/13 subjects with drug discontinuation in one subject after a week due to renal dysfunction. There was a significant reduction in mean PAdP after sacubitril/valsartan initiation compared to standard therapy (20.8 vs 18.3 mm Hg, p = 0.020). No further PAdP reduction was noted after sacubitril/valsartan dose increase (19.7 vs 20 mm Hg, p = 0.673) and at 3-month follow-up compared to baseline (20.8 vs 19.2 mm Hg, p = 0.352). CONCLUSIONS: Sacubitril/valsartan causes an acute reduction mean pulmonary artery pressures after initiation. However, no incremental reduction in PAdP was noted after dose increase and short-term follow-up. The current study demonstrates the utility of CardioMems™ device to study the drug's impact on hemodynamic profile in both short- and long-term follow-up.

Long-term use of the wearable cardioverter defibrillator in patients with explanted ICD.

OBJECTIVES: To evaluate the effectiveness of wearable cardioverter defibrillator (WCD) use in protecting patients from sudden cardiac arrest (SCA) while they were treated in nonhospital settings until re-implantation of an Implantable cardioverter-defibrillator (ICD) was feasible. We sought to determine whether the WCD could be successfully utilized long term (≥1 year) after ICD extraction in patients at continued risk of SCD in which ICD re-implantation was not practical. BACKGROUND: ICDs have proven to improve mortality in patients for both secondary and primary prevention of SCA. Increased ICD implantation in older patients with comorbid conditions has resulted in higher rates of cardiac device infections. Currently, a wearable cardioverter defibrillator (WCD) is an alternative management for SCA prevention in specific cases. METHODS: This a retrospective analysis based on consecutive WCD patients who underwent ICD explant due to device-related infections or mechanical reasons between April 2007 and July 2014. A total of 102 patients were identified from the national database maintained by ZOLL (Pittsburgh, PA, USA). We analyzed the reason for WCD use, demographic information, device data, compliance and duration of WCD use, detected arrhythmias and therapies, and reason for discontinuing WCD use. RESULTS: In these long term WCD users, average length of WCD use was 638 ±â€¯361 days. Nine patients (8.8%) had a sustained ventricular arrhythmia that was successfully resuscitated by the WCD. Six patients (5.8%) experienced inappropriate shocks. Two patients (1.9%) died of asystole events while wearing the WCD and an additional 10 patients died while not monitored by the WCD. Thirty-nine patients (38.2%) ended WCD use when a new ICD was implanted and 15 patients (14.7%) were still wearing the WCD at the time of analysis. CONCLUSIONS: We found that extending use of the WCD to ≥1 year is a safe and effective alternative treatment for patients with explanted ICDs who are not pacemaker dependent.

Cannabis induced asystole.

Cannabis or marijuana is the most used recreational, and until recently illegal, drug in the United States. Although cannabis has medicinal use, its consumption has been linked to motor vehicle accidents in dose dependent fashion. Marijuana and other cannabinoids produce a multitude of effects on the human body that may result in these motor vehicle accidents. Some of the effects that marijuana has been known to cause include altered sensorium, diminished reflexes, and increased vagal tone. We present a case of cannabis induced asystole from hypervagotonia.

Percutaneous closure of a left ventricular pseudoaneurysm in a high-risk surgical candidate.

Few cases of percutaneous device closure of a left ventricular pseudoaneurysm have been reported. We describe the case of a 67-year-old man with a history of coronary artery disease who presented with shortness of breath and chest pain. Computed tomographic angiography showed a left ventricular pseudoaneurysm that was filling from a small leak in the anterolateral aspect of the ventricle. The patient had undergone 3 previous sternotomies and was a high-risk candidate for surgical treatment of the pseudoaneurysm. Despite technical challenges, we closed the pseudoaneurysm percutaneously with use of a 6-mm AMPLATZER muscular ventricular septal defect occluder. The patient was released from the hospital the next day and was asymptomatic a year later.To our knowledge, this is the first report of the percutaneous closure of a left ventricular pseudoaneurysm via the femoral vein. We show that this manner of closure can be feasible in patients who have undergone multiple sternotomies and who are at high surgical risk.

Extracorporeal membrane oxygenation with hemoconcentration: in a hypervolemic patient with critical aortic stenosis.

Herein, we describe the case of a 60-year-old man with severe nonischemic cardiomyopathy and hypervolemia. By means of venoarterial extracorporeal membrane oxygenation at the bedside, along with hemoconcentration, the patient was resuscitated from severe cardiogenic shock and normal blood volume was restored. Within 24 hours, he was able to undergo a high-risk aortic valve replacement for severe aortic stenosis, with a successful outcome. To our knowledge, this is the first reported case in which hemoconcentration with extracorporeal membrane oxygenation has been used to support a patient with severe hypervolemia.

Left stellate ganglion block for continuous ventricular arrhythmias during percutaneous left ventricular assist device support.

A 58-year-old man presented with chest pain and tightness and was diagnosed with a Q-wave anterior myocardial infarction. He then developed pulseless ventricular arrhythmias, which were treated with repeated direct-current shocks and intravenous amiodarone. He underwent emergency cardiac catheterization: stents were deployed in the left anterior descending coronary artery and right coronary artery, and an intra-aortic balloon pump was inserted. Severe refractory cardiogenic shock and incessant ventricular arrhythmias compelled us to place a TandemHeart percutaneous left ventricular assist device 4 hours later. The patient's hemodynamic status stabilized, but the arrhythmias persisted for 36 hours. Multiple doses of intravenous amiodarone and lidocaine and multiple external direct-current shocks were all tried, but these measures failed to terminate the life-threatening ventricular arrhythmias. We performed a pharmacologic block of the left stellate ganglion, and this resulted in a return to sinus rhythm after 1 direct-current shock. To our knowledge, this is the 1st patient with refractory ventricular arrhythmias to have been treated with TandemHeart support and left stellate ganglion block.