Ambulatory trans-femoral intra-aortic balloon pump.

A 53-year-old man with inotrope-dependent advanced heart failure was admitted with acute decompensation and underwent urgent listing for heart transplant.

Sirolimus potentiated angioedema: A case report and review of the literature.

Introduction: In the realm of organ transplantation, particularly heart transplantation, angioedema presents a significant challenge. This clinical condition ranges from minor facial edema to life-threatening swelling of vital structures. Its multifactorial etiology involves various factors and mechanisms, including C1 esterase inhibitor deficiency, food allergen hypersensitivity, and adverse drug reactions, notably involving angiotensin-converting enzyme (ACE) inhibitors and mechanistic target of rapamycin inhibitors (mTOR-Is). We present a rare case of sirolimus potentiated angioedema in a patient with long-standing ACE inhibitor therapy. Case: A 52-year-old male with a history of heart transplant developed severe upper and lower lip edema. The patient had been on Lisinopril without any adverse events. However, sirolimus was recently added to his drug regimen. Sirolimus potentiated angioedema was suspected. Intervention: Intravenous methylprednisolone, famotidine, and diphenhydramine were initiated, and both lisinopril and sirolimus were discontinued. The patient showed improvement and was discharged with oral antihistamines. Lessons: Transplant physicians should be aware of the life-threatening interaction between ACE inhibitors and mTOR-Is like sirolimus. Consideration should be given to switching from an ACE inhibitor to an angiotensin-receptor blocker when initiating patients on mTOR-Is.

Right atrial pressure torsades de pointes.

A 39-year-old man with non-ischemic cardiomyopathy presented for routine right heart catheterization.

Intra-Caval Balloon Pump.

This clinical image vignette describes the inadvertent placement of a balloon pump in the vena cava during a "code blue" scenario, and the lessons to be learned from that experience. The hemodynamic benefits of intra-aortic balloon pump during experimental cardiac arrest include shorter circulation time and increases in end-tidal CO2 and coronary perfusion pressure. However, the hemodynamic effects of venous diastolic augmentation during experimental cardiogenic shock vary, being detrimental in cases of low preload, and possibly beneficial in a high preload state. When performed emergently at the bedside, inadvertent intra-caval placement of a balloon pump can occur due to elevated venous pressures, in the presence of severe tricuspid regurgitation, or through an arteriovenous fistula. A similar radiographic appearance can also be seen in the presence of a right-sided aortic arch. Clues to improper position include an abnormal pressure waveform and the absence of hemodynamic changes or blood pressure augmentation.

Percutaneous Treatment of Symptomatic Left Ventricular Assist Device Outflow Graft Obstruction.

A 60-year-old female underwent a left ventricular assist device implantation during which a 1-mm Gore-Tex sheet was used to cover the device and outflow graft to prevent future sternal re-entry injury. Seven years later, she developed low-flow alarms with a pattern of gradual decline in flow and power suggestive of outflow graft obstruction. After a discussion with the multidisciplinary team, percutaneous stenting of the outflow graft was pursued.

Sleeping prone with a left ventricular assist device: Dream or reality?

BACKGROUND: Among many other factors, restrictions on sleeping positions decrease quality of life after durable left ventricular assist device implantation. Specifically, device manufacturers and clinicians typically advise against sleeping in the prone position owing to concerns for driveline disruption, exit site infection, and hemodynamic compromise. Yet, no data exist to inform these recommendations and to the best of our knowledge no studies have examined sleep position in people with left ventricular assist devices. We report the first case of a person with a left ventricular assist device who slept prone, every night, for over one year. CASE: This is a 38-year-old man with a HeartWare™ left ventricular assist device who had previously not disclosed that he was sleeping in the prone position for the 16 months following device implantation. The patient did not suffer any episodes of device-related malfunction, infection, neurologic insult, or hemodynamic compromise. We performed a non-invasive assessment in both supine and prone positions to better understand the hemodynamic effects of the prone position. CONCLUSION: In select recipients of left ventricular assist devices, sleeping in the prone position may be safely tolerated.

Identifying Discordance of Right- and Left-Ventricular Filling Pressures in Patients With Heart Failure by the Clinical Examination.

BACKGROUND: In ≈25% of patients with heart failure and reduced left-ventricular ejection fraction, right-ventricular (RV), and left-ventricular (LV) filling pressures are discordant (ie, one is elevated while the other is not). Whether clinical assessment allows detection of this discordance is unknown. We sought to determine the agreement of clinically versus invasively determined patterns of ventricular congestion. METHODS: In 156 heart failure and reduced LV ejection fraction subjects undergoing invasive hemodynamic assessment, we categorized patterns of ventricular congestion (no congestion, RV only, LV only, or both) based on clinical findings of RV (jugular venous distention) or LV (hepatojugular reflux, orthopnea, or bendopnea) congestion. Agreement between clinically and invasively determined (RV congestion if right atrial pressure [RAP] ≥10 mm Hg and LV congestion if pulmonary capillary wedge pressure [PCWP] ≥22 mm Hg) categorizations was the primary end point. RESULTS: The frequency of clinical patterns of congestion was: 51% no congestion, 24% both RV and LV, 21% LV only, and 4% RV only. Jugular venous distention had excellent discrimination for elevated RAP (C=0.88). However, agreement between clinical and invasive congestion patterns was poor, к=0.44 (95% CI, 0.34-0.55). While those with no clinical congestion usually had low RAP and PCWP (67/79, 85%), over one-half (24/38, 64%) with isolated LV clinical congestion had PCWP <22 mm Hg, most (5/7, 71%) with isolated RV clinical congestion had PCWP ≥22 mm Hg, and ≈one-third (10/32, 31%) with both RV and LV clinical congestion had elevated RAP but PCWP <22 mm Hg. CONCLUSIONS: While clinical examination allows accurate detection of elevated RAP, it does not allow accurate detection of discordant RV and LV filling pressures.

Ascites and Edema After Bicaval Orthotopic Heart Transplant.

IVC stenosis is a rare complication of bicaval orthotopic heart transplant. IVC stenosis can occur at either the cavo-atrial anastomosis, or the caval cannulation site, with presentations ranging from acute shock early post transplant to a more indolent course. Causes include extensive hemostatic suturing, fibrous contraction, and donor-recipient size mismatch. Treatment strategies include percutaneous balloon angioplasty, stenting, and surgical revision. Evaluating for IVC stenosis is recommended for unexplained lower-extremity edema, new-onset ascites, or liver abnormalities after bicaval heart transplant.

A novel phonetic approach to continuous flow left ventricular assist device auscultation.

BACKGROUND: The dominant sound generated by continuous flow left ventricular assist devices (cf-LVADs) has generically been referred to as a "hum". This term, however, implies that despite distinct engineering designs, all cf-LVADs generate the same quality of sound. Furthermore, no structured method for auscultation of cf-LVADs exists. We hereby report a novel and simple phonetic approach to device auscultation, the aims of which are to improve recognition, understanding, and teaching of sound produced by normally functioning cf-LVADs. We sought to evaluate whether clinically relevant pump or person related events can produce changes in the expected audio fingerprint of a particular cf-LVAD, and whether these changes in sound can be identified by auscultation and translated phonetically. METHODS: Sound recordings were made on 7 people implanted with one of 3 U.S. Food and Drug Administration (FDA) approved cf-LVADs. Of the 7, 3 were considered to have normal device function and stable condition. The remaining 4 people had a clinically relevant pump or person related event. Recordings were made with a stethoscope attachment that allows digital recording of sound and provides a real time phonocardiogram. RESULTS: The main findings were the following: 1.) each cf-LVAD generates a unique audio fingerprint 2.) the audio fingerprint can be phonetically translated in a simple manner. 3.) pump or patient related events result in changes in device sound, that can be appreciated phonetically. CONCLUSIONS: Phonetics offer a novel and easily reproducible method for evaluation and teaching normal cf-LVAD sounds by auscultation. Additionally, clinically relevant pump or person related events can produce changes in the expected audio fingerprint of a particular cf-LVAD. These changes in sound can be identified by auscultation and translated phonetically. Auscultation is an important component of the physical examination of people supported with cf-LVADs.

Invasive Hemodynamic Red Flags to Left Ventricular Assist Device Implantation.

Reliable identification of patients at high risk for right ventricular failure is very important. We identify 4 parameters as hemodynamic red flags to left ventricular assist device implantation.

Heterozygous Cystic Fibrosis Transmembrane Regulator Gene Missense Variants Are Associated With Worse Cardiac Function in Patients With Duchenne Muscular Dystrophy.

Background Duchenne muscular dystrophy (DMD) is a neuromuscular disorder caused by mutations within the dystrophin gene. DMD is characterized by progressive skeletal muscle degeneration and atrophy and progressive cardiomyopathy. It has been observed the severity of cardiomyopathy varies in patients with DMD. Methods and Results A cohort of male patients with DMD and female DMD carriers underwent whole exome sequencing. Potential risk factor variants were identified according to their functional annotations and frequencies. Cardiac function of 15 male patients with DMD was assessed by cardiac magnetic resonance imaging, and various cardiac magnetic resonance imaging parameters and circulating biomarkers were compared between genotype groups. Five subjects carrying potential risk factor variants in the cystic fibrosis transmembrane regulator gene demonstrated lower left ventricular ejection fraction, larger left ventricular end-diastolic volume, and higher NT-proBNP (N-terminal pro-B-type natriuretic peptide) levels compared with 10 subjects who did not carry the potential risk factor variants (P=0.023, 0.019 and 0.028, respectively). Conclusions This study revealed heterozygous cystic fibrosis transmembrane regulator gene missense variants were associated with worse cardiac function in patients with DMD. The cystic fibrosis transmembrane regulator gene may serve as a genetic modifier that accounts for more severe cardiomyopathy in patients with DMD, who would require more aggressive management of the cardiomyopathy.

Cutaneous Vasculitis and Central Nervous System Infarctions due to Varicella Zoster Virus Vasculopathy in an Immunocompromised Patient.

Varicella zoster virus (VZV) infection commonly presents as varicella during childhood, and zoster, later in life. Here, we present a rare and interesting case of VZV infection that manifested with both cerebral and spinal infarctions and cutaneous vasculitis in the absence of a classic vesicular rash in an immunocompromised patient.