BACKGROUND: Alkaptonuria is a rare congenital metabolic disorder characterized by homogentisic acid accumulation in body cartilage and connective tissues due to a deficient homogentisic acid dioxygenase enzyme. This disorder manifests in various clinical symptoms, including spondyloarthropathy, ocular and dermal pigmentation, genitourinary tract obstruction by ochronosis stones, and cardiovascular system involvement. Cardiac ochronosis is a rare manifestation of alkaptonuria that may present as aortic stenosis, sometimes accompanied by other cardiovascular complications.
METHODS: We report an unexpected case of ochronosis diagnosed during cardiac surgery. Due to the fragile, thin, and atheromatous nature of the ascending aorta in patients with ochronosis, we opted for a sutureless aortic valve replacement procedure. This approach appears to be more suitable for patients with ochronosis.
CONCLUSIONS: Although cardiac ochronosis is rare, surgeons should remain vigilant and consider the possibility of this condition when examining patients with aortic valve stenosis, paying close attention to the clinical manifestations of alkaptonuria.

UNASSIGNED: Unicuspid aortic valve (UAV) represents a rare congenital anomaly characterized by two subtypes: acommissural unicuspid aortic valve and unicommissural unicuspid aortic valve. Acommissural UAV is often diagnosed and corrected during the neonatal period due to haemodynamic instability. Unicommissural UAV leads to aortic stenosis (AS) in early adulthood. The diagnostic challenge associated with UAV primarily stems from its eccentric orifice opening and valvular calcification, resulting in difficult visualization of the commissures and localization of the orifice plane. This case report aims to demonstrate the unique morphological features of UAV through a comprehensive analysis using multimodality imaging.
UNASSIGNED: A 61-year-old woman presented to the emergency department for recurrent episodes of dyspnoea. Severe AS was diagnosed on transthoracic echocardiography (TTE) by Doppler haemodynamic measurement. However, follow-up transesophageal echocardiography (TEE) and CT transcatheter aortic valve replacement showed moderate AS by planimetry. Following this, patient was monitored closely, but her dyspnoea kept worsening. Cardiovascular magnetic resonance (CMR) was performed due to persistent dyspnoea, identifying UAV with eccentric loophole orifice with unicommissural attachment and opposite free leaflet edge. The patient was managed medically.
UNASSIGNED: TTE is the test of choice for AS that defines valvular morphology by direct visualization and grades the severity by haemodynamic measurement. However, the accuracy of TTE can be limited by poor acoustic windows and heavy valvular calcification. TEE measures aortic valve area (AVA) by planimetry that requires accurate localization of the AV orifice plane. Similarly, it applies to multi-detector computed tomography (MDCT). While CMR is expensive and mainly available in tertiary centres, it can provide additional information when there is discordance.

Gallbladder perforation is a rare complication of acute cholecystitis that is associated with significant morbidity and mortality. Many cases of gallbladder perforation are not diagnosed until surgery, as the physical symptoms closely mimic acute cholecystitis. Gallbladder perforation is most common among older males with associated comorbidities, and preoperative assessment of comorbidities, particularly cardiac, is critical to determine the appropriate clinical course. We report a case of a 77-year-old male who presented initially with low blood pressure and right upper quadrant pain (RUQ) after not feeling well for five days. CT of the abdomen/pelvis with IV contrast demonstrated acute perforated cholecystitis, and general surgery was consulted for a cholecystectomy. Due to the patient\'s past medical history of severe aortic stenosis (AS), cholecystectomy was deferred and a cholecystostomy tube was placed by interventional radiology. This report aims to provide an example of a case of perforated cholecystitis with sepsis and how it can be diagnosed and managed non-surgically in the presence of pre-existing severe AS.

UNASSIGNED: There is a growing need for percutaneous coronary intervention (PCI) to be performed within the same transcatheter aortic valve implantation (TAVI) procedure. In such cases, cangrelor, a fast-acting intravenous P2Y12-inhibitor with a short offset, is potential clinical utility to minimize bleeding and vascular complications during large-bore arterial access (LBAA) as well as the thrombotic risk associated with concomitant PCI.
UNASSIGNED: We report two cases of TAVI with an indication to concomitant, high-risk PCI. In the first one, cangrelor was started only after LBAA was secured and TAVI completed, just before the initiation of complex PCI. In the second case, due to predicted complex coronary cannulation after TAVI, complex PCI was performed before TAVI and cangrelor started just after LBAA. In both cases, use of cangrelor (vs. pre-treatment with oral P2Y12-i) allowed for a tailored minimization of the risk of bleeding and vascular complications during LBAA while offering full platelet inhibition during a complex/high-risk PCI.
UNASSIGNED: In this case series, we illustrate a possible approach to the use of cangrelor for patients undergoing TAVI and complex/high-risk PCI. In such complex cases, thorough pre-procedural planning might include a cangrelor to minimize vascular, bleeding, and ischaemic complications.

UNASSIGNED: Percutaneous closure of aortic-to-right ventricle (ARV) fistula has emerged as an alternative to surgical management in selected cases. The use of three-dimensional (3D) printing in interventional planning for structural heart disease provides a concrete understanding, and it is useful in diagnostic assessment and to guide treatment approaches and to simulate procedures.
UNASSIGNED: We report a case of a 70-year-old male presenting in cardiogenic shock due to severe aortic stenosis and reduced left ventricular ejection fraction. The patient had several comorbidities and was deemed not eligible for cardiac surgery. After transcatheter aortic valve replacement (TAVR), an ARV fistula was discovered on the TTE. Due to complex anatomical considerations, a 3D printed model of the patient\'s anatomy was employed to supplement the decision-making process and technical planning of percutaneous ARV closure. Successful closure of the fistula with the use of the Amplatzer atrial septal occluder was subsequently performed.
UNASSIGNED: Three-dimensional printing improves the understanding of complex structures of cardiac diseases, allowing for enhanced planning and simulation of the procedure. This case, demonstrating the effective percutaneous closure of a TAVR-related ARV fistula facilitated by the use of 3D printed anatomical models in the pre-procedural phase, highlights the technology\'s potential in advancing patient-specific treatment approaches.

UNASSIGNED: Symptomatic aortic valve stenosis (AS) is associated with asymmetric basal septal hypertrophy (ABSH) in 10% of cases. In this cohort, it has been suggested that rectification of the left ventricular outflow tract obstruction (LVOTO) by concomitant septal myectomy (CSM) can improve the results of aortic valve replacement (AVR).
UNASSIGNED: This study aims to present the technique of AVR with CSM for severe AS with ABSH and to determine the associated early and late post-operative outcomes.
UNASSIGNED: Fifty-five patients were prospectively recruited to undergo AVR with CSM between 2011 and 2021 at two centres. The primary outcomes were mortality within 30 days, incidence of post-operative ventricular septal defects (VSD) and prosthetic valve sizing. The secondary outcomes were in-hospital complications, permanent pacemaker implantation (PPI), survival at 15 months and changes on transthoracic echocardiogram.
UNASSIGNED: Post-operative mortality was 1.8% and this figure was unchanged at 15-month follow-up. No patients developed a post-operative VSD. Intra-operatively, it was found that in 94.6% cases the direct valve sizing increased by one, when compared to the measurement made before CSM. The indexed effective orifice area (iEOA) was > 85 cm2/m2 in 96.4% and no patients had an iEOA ≤ 0.75 cm2/m2. Four patients (7.3%) required PPI due to complete atrioventricular block.
UNASSIGNED: AVR with CSM is a simple technique that can be utilised in severe AS with ABSH. There does not appear to be an increase in mortality or incidence of iatrogenic VSDs. Importantly, CSM allows for the implantation of a larger aortic valve compared to measurements made before CSM.

Aortic stenosis is the most common heart valve disease, especially among the elderly. Symptomatic aortic valve stenosis is linked to a poor prognosis and a high mortality rate if left untreated. The only effective treatment for severe symptomatic aortic stenosis is aortic valve replacement using either a mechanical or a biological prosthesis. Mechanical valve prostheses, while highly durable, are thrombogenic, necessitating lifelong anticoagulation with oral anti-vitamin K agents, such as acenocoumarol. Conversely, bioprosthetic valves, though less durable, carry a minimal thrombogenic risk and do not require anticoagulation. Currently, there is no proven role for direct-acting oral anticoagulants (DOACs) in patients with mechanical heart valves due to insufficient clinical trial data regarding their safety in this patient population. Herein, we present the case of a 59-year-old female known to have aortic stenosis, who underwent surgical treatment with mechanical aortic valve replacement eight years ago. Post-surgery, acenocoumarol was initiated. However, 18 months prior to presenting at our institution, the patient started taking rivaroxaban (a DOAC) instead of acenocoumarol due to the unavailability of acenocoumarol during the ongoing economic crisis in Lebanon, without consulting her cardiologist. Although she was followed up by her general practitioner and reported having a mechanical valve, her son contradicted this, claiming she had a biological valve. After thorough investigations, including chest X-ray, echocardiography, and fluoroscopy, it was confirmed that the patient indeed had a normally functioning mechanical aortic valve. Immediate corrective measures were taken, starting with IV unfractionated heparin and acenocoumarol, targeting an International Normalized Ratio (INR) between 2.5 and 3, while educating the patient about her condition and the importance of adhering to acenocoumarol therapy.

BACKGROUND: Premature aortic involvement and comprehensive management strategies in familial hypercholesterolemia familial hypercholesterolemia (FH), a rare autosomal dominant genetic disorder, poses significant challenges due to its propensity for elevated low-density lipoprotein cholesterol, premature coronary heart disease, and vascular atherosclerosis.
METHODS: Unraveling Cardiovascular Complexities: A Striking Familial Hypercholesterolemia. This case study delves into a remarkable instance of FH in a 16-year-old female who presented with chest pain and worsening dyspnea. Diagnostic evaluation revealed distinct electrocardiographic changes, elevated troponin levels, and profound dyslipidemia. Remarkable findings on transthoracic echocardiography, computed tomography angiography, and catheterization prompted multidisciplinary interventions and demonstrated remarkable enhancements in ventricular function, mitral regurgitation, and aortic stenosis.
CONCLUSIONS: The case study underscores the urgency of comprehensive management strategies in confronting the myriad challenges of FH, emphasizing the value of early intervention, innovative therapies, and rigorous imaging modalities for unraveling the intricate cardiovascular manifestations of this condition.

UNASSIGNED: The aetiology of secondary complete atrioventricular blocks includes ischaemia, cardiac sarcoidosis, electrolyte imbalance, drug use, rheumatic fever, and infections such as Lyme disease and endocarditis. Diagnosis is important since some of these causes are reversible. Although several studies have reported on aortic valve calcification causing complete atrioventricular blocks, no study has described improvement of complete atrioventricular blocks by removal of the calcification.
UNASSIGNED: A 42-year-old man with syncope had a Mobitz type II atrioventricular block, an alternating bundle branch block, and severe aortic stenosis. We identified a 10 s paroxysmal complete atrioventricular block with pre-syncope and performed pacemaker implantation. Electrocardiography-gated computed tomography confirmed that the calcification had reached the muscular septum. 18F-fluorodeoxyglucose-positron emission tomography (FDG-PET) revealed significant FDG uptake with high CT value of calcification in basal interventricular septum. The calcification in the septum was removed carefully, and aortic valve replacement was performed. The atrioventricular conduction capacity improved post-surgery. During the 1-year follow-up, the patient reported dramatic improvement in exercise capacity. We also noted an improvement of <0.1% in the right ventricular pacing burden.
UNASSIGNED: Complete atrioventricular blocks occur in patients with aortic stenosis accompanied by severe calcification of the aortic valve, which are visualized comprehensively by echocardiography. Electrocardiography-gated computed tomography and FDG-PET enabled detailed evaluation of the extent of calcification and pre- and post-operative tissue inflammation. Hence, we suspected that the calcification in the septum was causing complete atrioventricular block. Moreover, clinicians should recognize that aortic valve calcification with aortic stenosis can cause complete atrioventricular blocks.

UNASSIGNED: Cardiac resynchronization therapy (CRT) is recommended for patients with symptomatic heart failure in sinus rhythm with left ventricular ejection fraction (LVEF) ≤ 35%, QRS duration ≥ 150 ms, and left bundle branch block (LBBB) morphology. However, when severe left ventricular dysfunction and cardiogenic shock are present, treatment paradigms are often limited to palliative medical therapy or advanced therapies with durable left ventricular assist device or heart transplant as the functional and survival benefit of CRT in these patients remains uncertain.
UNASSIGNED: A 77-year-old white man with long-standing LBBB with dyssynchrony, severely reduced LVEF of 4%, and severe bicuspid aortic stenosis (AS) presented with worsening heart failure symptoms. After multidisciplinary heart team evaluation and pre-operative optimization, the patient underwent a surgical aortic valve replacement with simultaneous intraoperative initiation of CRT with pacemaker (CRT-P) and temporary mechanical circulatory support. Echocardiography at 44 days and 201 days post-discharge showed an LVEF of 29% and 40%, respectively.
UNASSIGNED: This case demonstrates that reverse remodelling and native heart recovery were successfully achieved in a patient with advanced structural heart disease, presenting with cardiogenic shock, through an early and aggressive approach involving multidisciplinary heart team evaluation, treatment of severe AS with surgical aortic valve replacement, prophylactic intraoperative initiation of temporary mechanical circulatory support, and early initiation of CRT-P.