Interruption of the Aortic Arch in the Adult and Fulminant Myocarditis: A Strange Presentation

Introduction

 
53 years old female patient, who presented oppressive precordial pain, radiating to the neck and jaw, for which she went to the emergency room, where an ECG image of complete left bundle branch block was evidenced (Figure 1), with taking ultrasensitive troponin I at > 50,000 pg/ml, presenting data of acute heart failure and acute pulmonary edema, requiring endotracheal intubation, moving urgently to cardiac catheterization without evidence of obstructive coronary lesions, observing in aortography interruption of the aortic arch with collateral vessels connecting to the descending aorta (Figure 2). Later in the ICU, an echocardiogram was performed that reported LVEF of 27%, generalized hypokinesia, severe MI and apical thrombus (Figure 3), diagnosing fulminant myocarditis, for which treatment with methylprednisolone at a dose of 500 mg for 3 days was started. presenting improvement with LVEF of 35%, however, she later presented septic shock of pulmonary origin, which led to the death of the patient. Aortic arch interruption is a congenital malformation characterized by complete interruption between the ascending and descending aorta, with 3 types according to the Celoria-Patton classification, B being the most frequent, while fulminant myocarditis is myocardial inflammation due to various etiologies , mainly viral, which can present from a picture of acute heart failure to cardiogenic shock and can simulate a picture of acute coronary syndrome. The importance of the previous case resides in the fact that, although there is no association between both pathologies, there is no case reported in the literature in which they occur simultaneously.

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Lupine Publishers | Non-Compacted Cardiomyopathy: Is there a Need of a New Cardiomyopathy?

Lupine Publishers | Journal of Cardiology & Clinical Research

 

Abstract

Left ventricular non-compaction (LVNC) is a myocardial disorder, classically defined as a double-layered myocardium, consisting of a thick, spongy/hypertrabeculated, non-compacted endocardial segment and a thin, compacted, epicardial portion. The American Heart Association (AHA) classifies LVNC as a distinct primary genetic cardiomyopathy, while the European Association of Cardiology (ESC) as an unclassified cardiomyopathy. Despite the magnitude of the entire literature yield on this topic, to date the pathogenesis, prognosis, and treatment are still unclear. Prevalence and mortality can range respectively from 0.05% to 0.26% and 5% to 47%, but they are affected by the imaging criteria adopted for the diagnosis. In fact, LVNC has been for years incidentally discovered during autopsy of unexplained sudden cardiac deaths. Conversely, with the advent of increasingly sophisticated cardiac imaging techniques, the presence of hypertrabeculated myocardium has become very common. Both echocardiographic and magnetic resonance criteria have been proven to overestimate the diagnosis, which shares a peculiar phenotype with other pathologies. It is known that a hypertrabeculated left ventricle leads to a symptomatic triad consisting of heart failure, arrhythmias and thromboembolisms. Therefore, it is current opinion of the authors that a “non-compaction cardiomyopathy” (NC-CMP) seems to be the most comprehensive definition of a disease that, similarly to the other cardiomyopathies, and regardless of its etiology, beyond a peculiar phenotype shares a distinct symptomatology and deserves to be listed as an entity between cardiomyopathies.

Keywords: Left Ventricle Non-Compaction; Non-Compacted Cardiomyopathy; Cardiomyopathies; Heart Failure; Sudden Cardiac Death

Introduction

Left ventricular non-compaction (LVNC) is a myocardial disorder, classically defined as a double-layered myocardium, consisting of a thick and spongy or hypertrabeculated endocardial segment, defined as non-compacted, and a thin and compacted portion, laying epicardially [1]. The above-mentioned hyper trabeculations (HXTs) characteristically involve the left ventricle (LV), especially the apex, the lateral, infero-lateral and inferior wall [1,2], and less frequently the right ventricle [3]. Since its first reports, LVNC has always been considered a controversial pathology (Figures 1 & 2). In facts Grant and colleagues, for long accredited as discoverers in 1926, presented a case of persistent sinusoids instead of LVNC [1]. In addition, a lack of uniqueness among the World Health Organization [4], the European Society of Cardiology [5]. and the American Heart Association [6] for its classification, and the presence of trabeculae as a terminal phenotype of LV hemodynamic overload or other myocardial affections, has led several authors to debate on the real existence of a true form of uncomplicated primitive non-compacted cardiomyopathy [7,8].

Figure 1: Two different anatomo-pathological macroscopic sections of the left ventricle, presenting a non-compacted myocardium. Modified from Lorca et al. Int J Cardiol 2016.

Figure 2: Echocardiographic features of a young adult of 16-years old, admitted in emergency room with acute signs and symptoms of acute heart failure.

Morpho Pathogenesis

The primitive hypothesis concerning LVNC is that the presence of HTXs is due to a failure in myocardial morphogenesis, in which the immature, non-compacted fetal myocardium normally undergoes a physiological compaction process during ontogenesis [1]. LVNC may occur isolated, in familial forms or associated with several congenital, genetic, neuromuscular and chromosomal conditions [9]. Mutations in the sarcomere gene, particular in MYH7, are the most common and non- sarcomere gene mutations (such as TAZ and NOTCH1) have also reported [10-12]. Some LVNC individuals have been detected by tracking asymptomatic relatives of affected patients [9], and therefore, a close correlation between genotype and phenotype has been recently underlined by 2 recent studies using NEXT-generation sequencing [13,14]. All these pathogenic variants were independent risk factors for cardiovascular events [13]. In addition, mutations in hyperpolarization-activated cyclic nucleotide channel 4 (HCN4) have also been reported in families with sinus node dysfunction and LVNC [15,16].

Epidemiology

Since only few studies focused on LVNC incidence, both prevalence and mortality are challenging to assess [1]. Among adults, it can be diagnosed in 0.05% - 0.26% of the cases, and approximately 0.14% of pediatric patient, with an overall mortality ranging from 5% to 47% in both populations [1]. Unfortunately, all the reports are affected by the diagnostic criteria adopted, imaging or autopsies, overestimating or underestimating the real prevalence [1,17] .In addition, and similarly with others cardiomyopathies, LVNC can be a subtle disease [17]; if not promptly diagnosed, patients may be asymptomatic for a long time and the onset may range from the early life to the adulthood [8].

Diagnosis

The advent of increasingly sophisticated cardiac imaging techniques set the spotlight on HXTs as a very common finding, instead of the rare disease that was previously considered [2,18,19]. Accordingly, Jenni, Chin and Stollberg defined different echocardiographic criteria, while Petersen, Jacquier, Captur and Stacey defined some cardiovascular magnetic resonance (CMR) criteria. 1 Unfortunately, they both showed very poor specificity [7,8], even if CMR overcame the ultrasound-related limits in morphologic assessment, and demonstrated a thigh correlation between late gadolinium enhancement myocardial fibrosis and clinical severity of the disease [20]. Nevertheless, there still a lack of an imaging-driven diagnostic gold standard [1,9].

Clinical Aspects and Treatment

Clinical findings are variable, including several grades of diastolic and systolic dysfunction, heart failure (HF), thromboembolic events, and malignant arrhythmias. 1 However, the most severe outcome is the sudden cardiac death. Atrial fibrillation, right/left bundle branch block, and repolarization abnormalities may be the only electrocardiographic features present at the moment of the diagnosis [2,8]. There is no specific therapy and LVNC management depends on the clinical manifestations; anticoagulation is indicated only if atrial fibrillation, heart failure, previous embolism, or intracardiac thrombus formation are present [1,21].

Discussion

LVNC has always been a controversial disease, with some unresolved issues. First of all, there is a heterogeneous genetic background and a wide spectrum of associated conditions that may occur contextually with HTXs. Secondly, LVNC real prevalence is still unknown. Certainly, the lack of any echocardiographic or CMR diagnostic gold standard, with frequent overestimation/ underestimation, does not help to clarify all the uncertainties. In addition, there are a wide variety of overlapping conditions that may occur with secondary myocardial HXTs, for example, a dilated cardiomyopathy or the end-stage hypertrophic cardiomyopathy (Figure 3). On the other hand, it is common experience that not all the above-mentioned cardiomyopathies and conditions can present an end-stage non-compacted phenotype and generalizing this aspect would be very simplistic. 8 In addition, Lorca and colleagues recently reiterated that non-compacted forms of cardiomyopathy do exist, especially during early stages of life, and they can be demonstrated in some forms of unexplained sudden deaths. 17 Furthermore, a multicenter longitudinal prospective study [7], despite its conclusions, and if carefully read between the lines, suggests that a significant proportion of asymptomatic patients meets all currently used imaging diagnostic criteria for LVNC.

Figure 3: Cardiac magnetic resonance imaging (CMR) exams of patients matching the currently imaging criteria for CMR (A, B, C, 2-chambers view; a, b, c, 4chambers view). (A, a) A 23-years-old male patients with a dilated cardiomyopathy. (B, b) a 38-years old woman with an end-stage hypertrophic cardiomyopathy. (C, c) a 45-years old woman admitted in emergency room for acute heart failure, and history of silent cerebral infarcts.

However, they demonstrated that outcomes are increased by symptoms and clinical conditions when associated with a noncompacted phenotype. Indeed, a recently published multicenter register provided an accurate estimation of genetic-phenotype association, and clinical and events of LVNC patients, concluding that the clinical course of symptomatic LVNC patient with a genotype-phenotype matching can be severe [13,14]. Given all these premises, it is reasonable considering the existence of a primitive non-compacted disease, congenital, and a mild form, with a late-onset, and/or acquired conditions. HF, thromboembolic events, and malignant arrhythmias seem to constitute the clinical triad for LVNC patients, and sudden cardiac death the most severe outcome. There is a tight genotype-phenotype correlation, with a wide spectrum of genes and mutation involved, as well as hypertrophic cardiomyopathy, for example, and the current imaging-derived diagnostic criteria probably need to be revised; perhaps it should be worth to combine some imaging and clinical criteria. At last, multicentric registries should be considered to help the real prevalence assessment of non-compacted forms of cardiomyopathies.

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Lupine Publishers | The Current Status of Continuous Flow Left Ventricular Assist Devices

Lupine Publishers | Advancements in Cardiology Research & Reports

Abstract

In this review, we hope to give a perspective of the new realities of cardiac mechanical circulatory assist devices. New iterations of devices are providing greater durability and freedom of complications. Work is near to provide internal batteries and transcutaneous energy transfer systems for completely implantable systems, avoiding the need for an externalized drive line.
Keywords: Heart Failure; Transplantation; Mechanical Circulatory Support

Twine and Twine or Lose the Plug- Dislodged Left Atrial Appendage Closure Device

In patients with severe heart failure, cardiac transplantation has been shown to provide considerable benefit. Since 1967, in excess of 88,000 total heart transplants have been performed and 1-year survival is 81%, the annual mortality is 4% per year thereafter. The supply of donor hearts is incredibly limited and much research has focused on mechanical means of improving myocardial function, and several such left ventricular assist devices (LVADs) have been developed through the National Institutes of Health artificialheart program. Several devices have been previously approved by the Food and Drug Administration as bridging therapy to transplantation, though none have been studied as long-term alternatives to transplantation. The Randomized Evaluation of Mechanical Assistance for the Treatment of Congestive Heart Failure (REMATCH) [1] trial explored whether a specific type of LVAD (a previous generation pulsatile device), when used in the long-term, would reduce mortality (Figure 1). The survival following severe heart failure was extremely poor in the optimally medically treated group in this trial (defined as End-stage heart failure was defined as New York Heart Association (NYHA) class IV symptoms for at least 90 days, left ventricular ejection fraction (LVEF) <25%, peak oxygen consumption <12 mL/kg/min or continued need for intravenous inotropes for symptomatic hypotension).
In the optimally medically treated control arm of the Randomized Evaluation of Mechanical Assistance for the Treatment of Congestive Heart Failure (REMATCH) trial [1] which evaluated an externalized pulsation ventricular assist device, survival at one year was 28% and 6% at two years, underlying the poor prognosis of this clinical entity (Figure 1). Driving the need for mechanical circulatory support (MCS) is the relative paucity of donors and the unmet need for orthotopic heart transplantation in the general population. There has also been an increase in the number of patients who require mechanical circulatory support (MCS) as a bridge to transplantation [2]. This has been driven, particularly in the UK by limitation of the number of hearts for donation, and also to buy time on the transplant waiting list. This is due to an increase in the numbers of non-heart beating donors (DCDs), whereby retrieval takes place in a circulation arrested donor, and the increased survival of head injury patients and those with intracranial bleeds who are treated by a decompressive craniotomy, reducing the pool of donors who have raised intracranial pressure and who have coned, resulting in brain stem death. The net result is a retrieval rate for heart transplantation of around 19%. The risk of having preformed antibodies directed against the donor heart (sensitised patients) is increasingly likely and is particularly challenging as it may increase the risk of rejection and allograft vasculopathy. There has also been an increase in the number of patients requiring MCS as a bridge to transplantation [3]. This allows many severely ill adults and paediatric patients to survive until a suitable donor heart is available. Patients with MCS are at increased risk for rejection, infection, stroke, and bleeding. The need for transfusions also increases the risk of pre-sensitization [3-5]. Survival at 1 and 5 years is decreased in patients requiring MCS prior to transplantation, but still higher than 80% and 70%, respectively (ISHLT database) [2].

Figure 1: Randomized Evaluation of Mechanical Assistance for the Treatment of Congestive Heart Failure (REMATCH) trial (Rose et al N Engl J Med 2001; 345:1435-1443).

Advances in Donor Allocation and Selection

Recipient criteria for heart transplantation include, severe symptoms despite maximal medical management, the absence of reversible or surgically amenable heart disease, and where estimated 1-year survival is less than 50% [6]. An estimate of functional capacity in ambulatory patients can be best quantified by measurement of peak O² consumption (VO²max). Patients with low VO2max (<12 ml/min/kg) have high mortality even if treated with beta blockers and transplantation should be considered for these patients. In addition, heart failure prognosis scores to estimate survival, such as the Heart Failure Severity Score may be used. This calculates a survival probability on the basis of the presence of ischaemic cardiomyopathy, resting heart rate, left ventricular ejection fraction, mean blood pressure, interventricular conduction delay, VO²max and serum sodium concentration [7].

Figure 2: Competing outcomes for continuous flow LVADS (82% survival at 1 year, intention to treat).

Transplantation eligibility is always considered with regard to risk factors, especially, pulmonary hypertension (Figure 2). Right heart catheterization must be performed in all potential candidates for heart transplantation in order to quantify pulmonary vascular resistance [7]. Right heart failure is a substantial cause of mortality. Right ventricular failure is likely when post implant pulmonary artery pressures exceed 50 mmHg. Patients with chronic heart failure may develop pulmonary hypertension due to elevated left ventricular end diastolic pressure with elevated left atrial and pulmonary venous pressures. This is a reactive form of pulmonary hypertension and may fall when the cardiac output is increased with inotropes or unloaded with nitrate infusions [7]. The transpulmonary gradient is calculated by subtracting the left atrial filling pressure from the mean pulmonary artery pressure. A fixed transpulmonary gradient in excess of 14 mmHg is associated with greatly elevated risk, and thus this cut off is used in the UK [8]. In such patients a destination therapy strategy may be used with continuous flow LVADS.

Mechanical Circulatory Assist Devices

In recent years, the use of MCS device in treating patients with end-stage heart disease has increased significantly, as bridge to transplantation and as destination therapy for transplant ineligible candidates. This increase is based on the accumulated experience with new second-generation continuous-flow devices which show significant improvements in survival, functional capacity and quality of life [9,10]. On the basis of the Heart Mate II Registry experience (1300 patients), guidelines for the clinical management of patients treated with continuous-flow devices have been published [11]. Risk scoring systems, such as the Seattle Heart Failure Model [12] and the Cumulative Risk Score for 90-Day in-Hospital Mortality [13] and the Destination Therapy Risk Score have been investigated to stratify patients who might benefit from LVAD support [14].
Right ventricle failure is a leading cause of morbidity and death after LVAD implant (incidence of about 35%), and can be very difficult to predict [15,16]. Various means to assess right ventricle function both pre- and postoperatively have been assessed (10). Right ventricular failure risk scores have been created that stratify the risk of right ventricular failure (RVFRS) and death after LVAD implantation (Figure 3). One such RVFRS found independent predictors of right ventricular failure to include vasopressor requirement, aspartate aminotransferase >80 IU/L, bilirubin >2.0mg/dL and creatinine >2.3mg/dL [15]. Another study developed a score to predict RVAD need after LVAD placement, which included factors of cardiac index, right ventricular stroke work index, severe preoperative right ventricular dysfunction, creatinine, previous cardiac surgery and systolic blood pressure [16]. More recently the presence of severe TR and a tricuspid annulus of >43mm and right ventricular sphericity have been proposed as predictive of occult RV failure and need for biventricular support. The Interagency Registry for Mechanically Assisted Circulatory Support (INTERMACS) registry, which follows all long-term MCS systems in the United States, has defined patient profiles that can help identify risks associated with the timing of implant [17]. In the future, the INTERMACS patient profile would be a useful tool to improve management and outcomes of patients who need VAD implant and unify criteria for future clinical trials and devices (Figure 4). As more LVAD patients are listed for heart transplant, a competition has occurred for organs between stable LVAD supported registrants and less stable registrants listed UNOS status 1A or 1B (the highest categories and most at risk if not urgently transplanted). A recent study found that stable LVAD patients had significantly less 30-day risk of events compared to other status 1A patients concluding that allowance of 30 days of elective status 1A time should not be allocated to stable registrants with implanted LVADs [18]. As VAD technology improves, further revisions to the allocation system will need to be recommended.

Figure 3: Heartmate 3, the latest centrifugal blood pump in comparison to Heartmate II an axial flow pump. Superior event free survival is seen with HM 3.

Figure 4: Heart-mate 3 vs Heartmate II comparison of event free survival.

INTERMACS Profile and Description and Timescale to MCS
a) “Crashing and burning”—critical cardiogenic shock. Within hours
b) “Progressive decline”—inotrope dependence with continuing deterioration. Within a few days
c) “Stable but inotrope dependent”—describes clinical stability on mild-to-moderate doses of intravenous inotropes (patients stable on temporary circulatory support without inotropes are within this profile). Within a few weeks
d) “Recurrent advanced heart failure”—“recurrent” rather than “refractory” decompensation. Within weeks to months
e) “Exertion intolerant”—describes patients who are comfortable at rest but are exercise intolerant. Variable
f) “Exertion limited”—describes a patient who is able to do some mild activity but fatigue results within a few minutes of any meaningful physical exertion. Variable
g) “Advanced NYHA III”—describes patients who are clinically stable with a reasonable level of comfortable activity, despite history of previous decompensation that is not recent. Not a candidate for MCS
INTERMACS = Interagency Registry for Mechanically Assisted
Circulatory Support; MCS = mechanical circulatory support;
NYHA = New York Heart Association.

Figure 5: Durability of HM 3 vs HM II, freedom from pump replacement due to pump thrombosis or haemolysis.

Temporary MCS are available that can be implanted quickly and simply to normalise cardiac output in patients with severe acutely decompensated heart failure. The CentriMag [19], Tandem Heart [20], Impella [21] and Circulite [22]. Clinical trials suggest that treatment of temporary VADs does not necessarily correlate with better survival, but merely comprise a component of treatment leading to recovery, upgrade to fully implantable systems as a bridge to transplant or destination therapy, or transplantation [23,24]. Device miniaturisation, without externalized drive-lines connecting the device to a console and longer endurance will be the future trend of mechanical design for long term support. Blood pumps with magnetically levitated rotors has shown satisfactory 1-year survival [25]. The smaller size and weight of the continuousflow devices has allowed an extension of the new VADs into smaller patients. Fully wireless resonant coupling power sources are currently undergoing evaluation, which if successful will greatly reduce the incidence of drive line infections (Figure 5), which is the weakest point of the technology of current fully implantable systems. There is some evidence that fully implantable systems will be available in the near future to greatly improve the quality of life and to reduce the frequency of severe infections with continuous flow LVADS.
Many recent studies have focused on the reversed molecular and cellular alterations, such as improved β-adrenergic responses and decreased calcium-regulating gene expression (Figure 6), in patients using LVAD as a bridge to recovery therapy [26]. Functional recovery has been observed in a subset of heart failure patients [26,27]. Recently, a clinical trial using clenbuterol (β-2 agonist and anabolic agent) and LVAD in refractory non-ischemic heart failure patients, reported recovery of heart function in 60% of patients (n=20) with non-ischemic cardiomyopathy that allows the pump to be explanted (Harefield Recovery Protocol Study for Patients with Refractory Chronic Heart Failure, HARPS) [28]. LVAD therapy is associated with decreased collagen turnover and crosslinking and increased tissue angiotensin II. LVAD combined with angiotensinconverting enzyme inhibition results in decreased tissue angiotensin II and collagen cross-linking, normalizes left ventricular end-diastolic pressure volume relationships and is associated with modestly higher rates of bridge to recovery [29]. Other adjunctive treatments including other medications, cell or gene therapy with over expression of SERCA2a might in conjunction with VAD support provide a meaningful alternative therapy in patients with severe heart disease [30].

Figure 6.

Conclusion

Heart transplantation is associated with excellent long-term outcomes and is the gold standard solution for intractable end stage heart failure in eligible patients. What limits its impact, overall, is the limited availability of donor organs. The development of ventricular assist devices has mitigated against this, to some extent. Subsequent device iterations with further miniaturisation and continuous flow have resulted in effective bridge to transplant solutions. The presence of an externalized drive line exposes the VAD recipient to infections, however, which may precipitate urgent listing for heart transplant in the bridge to transplant candidate and may limit the life span of the destination therapy candidate. Fully implantable driveline free systems will definitely enhance the utility of these systems in these settings. As our knowledge of molecular medicine increases, manipulation of key proteins implicated in the pathophysiology of heart failure such as SERCA2a may allow some recovery of the myocardium in patients with heart failure to the extent that transplantation may be deferred or the LVAD explanted [31-35].

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