Biatrial Dilatation Research Articles

Miocardiopatía restrictiva

Restrictive cardiomyopathy (RCM) is the rarest form of cardiomyopathy and must be included into the pathophysiology of diastolic dysfunction. Restriction leads to significant rise in left- and rightheart filling pressures causing a systemic and pulmonary venous congestion. Restrictive cardiomyopathy (RCM) is characterized by normal-sized ventricles, marked biatrial dilation, diastolic restrictive pattern and normal or near normal systolic function. MCR shows very similar symptomatology and hemodynamic characteristics to chronic constrictive pericarditis, being necessary surgical exploration in some cases. It is an uncommon cardiomyopathy in the Western world, being amyloidosis and idiopathic cases the most frequent clinical entities. Clinical findings are nonspecific and usually resemble to those observed in heart failure. Occasionally, rhythm disturbances and severe arrhythmias are observed. Echocardiography is the diagnosis test of choice.

325 * TOTALLY ENDOSCOPIC SURGICAL EPIMYOCARDIAL ABLATION IN PATIENTS TURNED DOWN FOR CATHETER TREATMENT DUE TO PERSISTENT LONE ATRIAL FIBRILLATION AND SIGNIFICANT ATRIAL DILATATION

Objectives: To analyse the safety and efficacy of surgical endoscopic epimyocardial ablation in patients turned down for interventional catheter therapy due to long-standing persistent atrial fibrillation (AF) combined with significant atrial dilatation (>5 cm). Methods: Since December 2010, 15 patients with no reasonable interventional therapy option were referred by electrophysiology for surgical ablation (persistent AF + biatrial dilatation [left atrial diameter 5.8 ± 0.6 cm]). The mean patient age was 52 ± 6 years, 66.6% were male, body mass index 38 ± 6, AF duration 2.8 ± 1.2 years, left ventricular ejection fraction 46 ± 12%, and left ventricular end diastolic diameter 54 ± 6 mm. Ablation was performed via a bilateral endoscopic approach by bipolar radiofrequency. All patients received intra- and postoperative amiodarone for three months, and monitoring was achieved by an implanted event recorder. The primary endpoint was cardiac rhythm after 12 months. Results: All patients successfully received bilateral pulmonary vein isolation + box lesion + trigonum lesion + left atrial appendage resection. Successful isolation was confirmed by intraoperative mapping followed by conversion to sinus rhythm (SR). The mean procedure duration was 235 ± 70 min. There was no intraoperative complication; one patient shows persistent left phrenic nerve palsy. The mean hospital stay was 4 ± 2 days, and mean follow-up 18 ± 6 months. The incidence of SR was 67%, 73% and 80% at discharge, and at 3- and 12-month follow-up respectively. Two additional patients were free from AF, but developed paroxysmal right atrial tachycardia which was successfully treated by catheter intervention at three and four months postoperatively. All patients in SR (14/15, 93%) are off anticoagulation and anti-arrhythmic drugs. Conclusions: Totally endoscopic complete left atrial ablation, including left atrial appendage resection, can be safely performed even in this difficult patient population. It achieved excellent rates of SR restoration in patients with long-standing persistent AF combined with significant atrial dilatation, particularly if combined with additional interventional follow-up.

AL amyloid arthropathy and cardiomyopathy presenting with progressive symmetrical fixed flexion deformities and new onset congestive heart failure

Dear Editor, A 73-year-old man was admitted with new-onset congestive heart failure and renal dysfunction. He had a 1–2 year history of a symmetrical deforming polyarthropathy characterized by persistent stiffness and progressive fixed flexion deformities, particularly of the fingers and knees. Examination revealed cachexia and generalised symmetrical muscle wasting. Bony swelling was present in the small and large joints with firm non-tender prominences over the volar aspect of both wrists. There was no clinical evidence of synovitis. X-rays of the wrists revealed significant soft tissue swelling with erosions and extensive subchondral cysts within the carpi, adjacent radii and ulna (Fig. 1). Joint spaces were preserved with only subtle subchondral sclerosis. A transthoracic echocardiogram revealed a restrictive cardiomyopathy (ejection fraction 26%). Cardiac magnetic resonance imaging (Fig. 2a) demonstrated increased biventricular and biatrial wall thickness (especially of the atrial septum), biatrial dilatation and bilateral pleural effusions (*). There was global, “ring-like” subendocardial hyperenhancement in the late gadolinium enhancement images (Fig. 2b; arrows; RV, right ventricle; LV, left ventricle). An endomyocardial biopsy revealed homogenous eosinophilic material within blood vessel walls and the interstitium, surrounding individual myocytes with associated myocyte atrophy. This stained positively for Congo red with apple-green birefringence under polarised light (Fig. 3). Serum electrophoresis was unremarkable. A serum free light chain ratio was at the upper limit of normal considering his renal function (kappa:lambda 3.12). Urine electrophoresis demonstrated trace monoclonal kappa free light chain making AL amyloidosis the most likely diagnosis. A bone marrow biopsy demonstrated a slight plasmacytosis (4%) with a high proportion of cells expressing kappa light chains (10%). His serum uric acid was 0.58 mmol/L, troponin T 0.163 μg/L and N-terminal pro-brain natriuretic peptide (NT-proBNP) 15 926 ng/L, giving an estimated prognosis of 4 months.1 A palliative approach was pursued. Although no biopsy was performed of his periarticular cysts, amyloid light-chain arthropathy was felt to be the most likely aetiology of his joint disease.

Giant thrombosed saccular aneurysm of the ascending aorta

A 74-year-old woman with a history of hypertension, heart failure, asthma, and amputated right arm (secondary to arterial thromboemboli) was admitted to the emergency department with complaints of chest distress and dyspnea. She was normotensive and her heart rate was 76 bpm. On the physical examination, bilateral rales up to the mid zones of the lungs and grade III systolic murmur at the left sternal border and apex were heard. Electrocardiography showed QS pattern in anterior precordial derivations, and troponinI level was 1.0 ng/ml (reference range: 0-0.6 ng/ml). Transthoracic echocardiography revealed valvular insufficiencies (moderate-to-severe tricuspid regurgitation, moderate mitral regurgitation and mild aortic regurgitation), biatrial dilatation, left ventricular hypertrophy, and systolic dysfunction (ejection fraction [EF] was 40%, with segmental wall motion abnormalities at the apex and apical segments). On chest X-ray, congested lungs, increased cardiothoracic ratio, and dilated-calcified ascending and arcus aorta were detected. After admission to the coronary care unit, coronary angiography was performed, and a giant thrombosed saccular aneurysm of 120x50 mm was visualized (Fig. A, B). The aneurysm was limited to the ascending aorta (Video*). In addition, we detected several critical stenoses in coronary arteries. Although this giant aneurysm was detected incidentally, it is the most likely cause of her previous thromboemboli to the right arm that resulted in amputation. This giant aneurysm went undetected because the patient did not undergo computed tomography or conventional angiography during the first event. Because of the giant saccular aneurysm and multivessel disease of the coronary arteries, surgery was decided for the patient. As the patient refused surgery, no tomographic imaging was performed and she was discharged with medical therapy. 781

Autosomal Recessive Atrial Dilated Cardiomyopathy With Standstill Evolution Associated With Mutation of Natriuretic Peptide Precursor A

Atrial dilatation and atrial standstill are etiologically heterogeneous phenotypes with poorly defined nosology. In 1983, we described 8-years follow-up of atrial dilatation with standstill evolution in 8 patients from 3 families. We later identified 5 additional patients with identical phenotypes: 1 member of the largest original family and 4 unrelated to the 3 original families. All families are from the same geographic area in Northeast Italy. We followed up the 13 patients for up to 37 years, extended the clinical investigation and monitoring to living relatives, and investigated the genetic basis of the disease. The disease was characterized by: (1) clinical onset in adulthood; (2) biatrial dilatation up to giant size; (3) early supraventricular arrhythmias with progressive loss of atrial electric activity to atrial standstill; (4) thromboembolic complications; and (5) stable, normal left ventricular function and New York Heart Association functional class during the long-term course of the disease. By linkage analysis, we mapped a locus at 1p36.22 containing the Natriuretic Peptide Precursor A gene. By sequencing Natriuretic Peptide Precursor A, we identified a homozygous missense mutation (p.Arg150Gln) in all living affected individuals of the 6 families. All patients showed low serum levels of atrial natriuretic peptide. Heterozygous mutation carriers were healthy and demonstrated normal levels of atrial natriuretic peptide. Autosomal recessive atrial dilated cardiomyopathy is a rare disease associated with homozygous mutation of the Natriuretic Peptide Precursor A gene and characterized by extreme atrial dilatation with standstill evolution, thromboembolic risk, preserved left ventricular function, and severely decreased levels of atrial natriuretic peptide.

AA-Amyloidose mit kardialer Beteiligung

A 51-year-old woman was referred for kidney transplant evaluation. An HIV infection had been diagnosed ten years ago, viral load being negative at present. Renal type AA-amyloidosis was demonstrated by kidney biopsies. The patient had no symptoms. Clinical examination revealed no significant abnormalities with exception of a systolic murmur. A 12-channel electrocardiogram demonstrated peripheral low voltage. Echocardiography showed severe diastolic dysfunction, ventricular hypertrophy and biatrial dilatation. In addition, the aortic valve displayed a calcified mass. Treatment with an ACE-inhibitor for diastolic heart failure was continued, and the patient was included into the renal transplantation program. Amyloidosis is characterized by an abnormal folding of certain proteins and their extracellular deposition in several organs. This leads to corresponding dysfunction and can result in organ failure. Cardiac involvement is usually associated with immunoglobulin-light-chain (AL) - or familial transthyretin-associated (ATTR) - amyloidosis and indicates a poor prognosis. This report demonstrates a rare case of pronounced cardiac manifestation of the amyloid protein A (AA) - amyloidosis.

The chance finding of echocardiographic complications of infective endocarditis

Fig. 1. Biatrial dilatation, a left ventricular dilatation, an interventricular septal hypertrophy, an anterior mitral leaflet abscess/perforation complication of infective endocarditis. Despite the progress in its knowledge and treatment, infective endocarditis [1–17] remains a therapeutic challenge [17]. We present a case of an infective endocarditis in a 62-year-old Italian man. A 62-year-old Italian man was admitted to the Cardiology Unit for a pre-operative cardiac risk assessment in colon cancer surgery. He reported a streptococcal infection two years ago. Echocardiographic evaluation revealed a biatrial dilatation and a left ventricular dilatationwith an interventricular septal hypertrophy. The semilunar aortic valves were markedly thickened with retraction of left and right cusps. A moderate–severe mitral regurgitation, a severe aortic regurgitation, a mild tricuspidal regurgitation, and an anterior mitral leaflet abscess/perforation complication of infective endocarditis were also observed. Also this case focuses attention on infective endocarditis.

Updates in Cardiac Amyloidosis: A Review

Systemic amyloidosis is a relatively rare multisystem disease caused by the deposition of misfolded protein in various tissues and organs. It may present to almost any specialty, and diagnosis is frequently delayed.[1][1] Cardiac involvement is a leading cause of morbidity and mortality, especially

A case of restrictive cardiomyopathy evaluated by cardiac magnetic resonance imaging

A 66-year-old woman, known to have hypertension, hypothyroidism, and atrial fibrillation for the last 8 years, presented with ankle swelling, abdominal distention, and worsening of breathlessness for the last 4 months. She was evaluated and found to have features of heart failure on clinical examination. Two-dimensional (2D) echo revealed global hypokinesia with left ventricular (LV) dysfunction and mild pericardial effusion. The cardiac magnetic resonance imaging study showed all the characteristic features of amyloidosis (Figure 1). Figure 1 (A) Biatrial dilatation with normal sized ventricles. (B) Concentric left ventricular hypertrophy (LVH) with mild pericardial effusion. (C) There is late gadolinium enhancement in the IAS and the entire subendocardium of the LV not conforming to any coronary ... The delayed gadolinium kinetics was highly suggestive of amylodosis.

POSTER PRESENTATIONS

POSTER PRESENTATIONS

Cardiac and muscle imaging findings in a family with X-linked Emery–Dreifuss muscular dystrophy

The following is a report on a large family with 5 males affected by the X-linked recessive form of Emery–Dreifuss muscular dystrophy with mutation in the STA gene. A detailed longitudinal cardiological evaluation and muscle imaging studies allowed for the assessment of intrafamilial variability of cardiac and muscle involvement.Long term cardiological follow up in the 5 affected males and in 7 female carriers revealed different degrees of severity, ranging from tachycardia–bradycardia syndrome and variable biatrial and left ventricle dilatation, to an episode of isolated symptomatic sustained ventricular tachycardia requiring a device implantation.Muscle imaging in the affected males showed involvement of the soleus and medial head of gastrocnemius on leg muscles and variable involvement on thigh muscles that have not been previously reported. In some cases, imaging showed clear signs of muscle involvement even when no overt signs of weakness could be detected during clinical examination.

Idiopathic biatrial dilatation characterized by multimodality imaging

Idiopathic biatrial dilatation characterized by multimodality imaging

Idiopathic biatrial dilatation characterized by multimodality imaging

Idiopathic biatrial dilatation characterized by multimodality imaging

Imaging Phenotype Versus Genotype in Hypertrophic Cardiomyopathy

Hypertrophic cardiomyopathy (HCM) is a common monogenetic cardiac disease with a prevalence of 0.2%1 that usually is inherited as an autosomal dominant trait with variable penetrance and expression. Clinically, HCM is defined by the presence of left ventricular (LV) hypertrophy (LVH) unexplained by abnormal loading conditions.1 The natural history varies from an asymptomatic course to drug-refractory angina/dyspnea, sudden cardiac death (SCD), and end-stage heart failure. The incidence of HCM-related SCD is approximately 1% to 2% in children and adolescents and 0.5% to 1% in adults.2,3 In this review, we examine the impact of advanced diagnostic imaging technologies on HCM, with particular emphasis on the detection of particular genetic subtypes. In ≈60% of cases, HCM is inherited as an autosomal dominant trait caused by mutations in genes coding for cardiac sarcomere proteins (Figure 1).4–7 Mutations in genes encoding Z-disc proteins and proteins involved in calcium regulation account for <5% of cases. The absence of sarcomeric gene mutations can be explained by limitations of current mutation detection techniques or mutations in as yet unidentified genes, but in some cases, hypertrophy is caused by other diseases that mimic the phenotype of sarcomeric protein disease. Table 1 lists genetic disorders associated with HCM. Figure 1. A schematic illustration of a cardiac sarcomere. View this table: Table 1. Currently Known Genes Implicated in HCM More than 600 different sarcomeric gene mutations are reported in patients with HCM.8–16 The majority are missense mutations, but nonsense, frameshift, and in-frame insertion/deletion mutations also occur. Most mutations are characterized by incomplete penetrance and variable clinical expression likely due to locus heterogeneity and the effect of mutations at different locations within the same gene. Most mutations probably have a dominant negative effect on sarcomere function, but in some cases, haploinsufficiency (ie, only a single functional …

E0233 Late gadolinium-enhanced MRI in restrictive cardiomyopathy

ObjectiveTo evaluate the diagnostic value of MRI in combination of late gadolinium-enhanced imaging in the identification of restrictive cardiomyopathy (RCM).Methods116 patients with RCM underwent ECG, x-ray, Echocardiography and MRI. The...

E0699 Late gadolinium-enhanced cardiac MRI in restrictive cardiomyopathy

ObjectiveTo evaluate the diagnostic value of MRI in combination of late gadolinium-enhanced imaging in the identification of restrictive cardiomyopathy (RCM).Methods116 patients with RCM underwent ECG, X-ray, Echocardiography and MRI. The...

Reply: Bevacizumab reverses need for liver transplantation in hereditary hemorrhagic telangiectasia

We would like to thank Buscarini and colleagues1 for their interest in our use of bevacizumab in a patient with severe hereditary hemorrhagic telangiectasia (HHT) with life-threatening hepatic involvement and for allowing us to provide follow-up information. Our patient was on maximal medical therapy for her high-output cardiac failure, portal hypertension, and ascites. Failure of standard medical therapy had led to referral for consideration for liver transplantation. Specifically, prior to the trial of bevacizumab, she was on fluid and sodium restriction, propanolol (40 mg 3 times a day), spironolactone (400 mg daily), and furosemide (40 mg twice daily). Higher doses of diuretics had been limited by renal impairment and electrolyte imbalance. Anemia was corrected with iron infusions and transfusions of packed cells with some difficulty, as it precipitated fluid overload. Her hemoglobin was 100 g/L at the time of her first bevacizumab infusion. At the end of her bevacizumab infusions, all medications were able to be stopped. We agree that HHT patients fit to be considered for liver transplantation should undergo right heart catheterization if noninvasive techniques suggest ventricular failure and/or high pulmonary arterial pressures. Right heart catheterization was not performed in our patient as she was considered too unwell for transplantation. At the time of presentation, her echocardiogram showed moderate to severe biventricular and biatrial dilatation, with an estimated pulmonary arterial systolic pressure of 41 mm Hg. Magnetic resonance cardiography revealed right and left ventricular ejection fractions of 54% and 62%, respectively, and right and left ventricular cardiac outputs of 10.4 and 10.2 L/minute, respectively. She remained in sinus rhythm throughout. After her dramatic response to the first course of bevacizumab, the patient remained stable and asymptomatic for 9 months. At this time, she redeveloped mild ascites associated with weight gain, tachycardia, and increased epistaxis. She began to experience breathless walking on flat ground, whereas previously she had been able to kayak for several kilometers. Her symptoms were associated with an increase in hepatic volume and cardiac output (see Fig. 1). Spironolactone and propranolol were reinstituted without improvement. After discussion with the patient, a second course of 6 infusions of bevacizumab was given. This was again tolerated well apart from minor mouth ulceration. By the end of the second course, her ascites had again disappeared, and the cardiac and hepatic volumes had fallen back to posttreatment values of the first course. Again, she was able to cease her medications. Seven months after the completion of the second course of bevacizumab, the patient remains asymptomatic and off medications. Six months after her second course, her cardiac output and hepatic volumes have increased slightly; however, they remain significantly lower than their baseline values. At this stage, we have continued to monitor her closely and have not treated her further, given that she is asymptomatic. Response of the liver volume and cardiac output to bevacizumab therapy. The role of bevacizumab in the management of patients with HHT cannot be established on the basis of our single case report, nor is it a replacement for liver transplantation. As Buscarini and colleagues1 point out, increased toxicity has been found in phase 3 trials in which bevacizumab was added to standard chemotherapy in patients with nonresectable colorectal or lung malignancy.2, 3 In particular, mucocutaneous hemorrhage, including grade 1 epistaxis, may be a specific concern in HHT patients, although our patient noted a reduction in her epistaxis. In addition, bevacizumab has been associated with poor wound healing, although it has not been reported to be associated with increased surgical complications when given after a perioperative window of 28 days.4 Alternative therapies, including hepatic arterial ligation or embolization and transplantation, have considerable toxicity of their own. Up to 39% of HHT patients undergoing hepatic artery ligation or embolization have complications that lead to death or requirement for liver transplantation,5 which in itself has a perioperative mortality rate of 18%.6 Given the dramatic response to bevacizumab, we strongly feel that bevacizumab should be considered for patients too ill to undergo transplantation. Its role in symptomatic patients suitable for transplantation is unclear, and further experience in a clinical trial setting is required to clarify this. Lastly, it is exciting to ponder that vascular endothelial growth factor antagonists may have a role in reducing symptoms in the substantial number of HHT patients who are symptomatic but do not require liver transplantation. We agree with Buscarini et al.1 that only with time will the full clinical role of bevacizumab in symptomatic HHT be made clear. Leon A. Adams* , Andrew Mitchell*, Gerry MacQuillan* , Jonathon Tibballs , Rohan vanden Driesen , Luc Delriviere , * Western Australian Liver Transplant Service, Sir Charles Gairdner Hospital, Nedlands, Australia, Department of Radiology, Sir Charles Gairdner Hospital, Nedlands, Australia, School of Medicine and Pharmacology, University of Western Australia, Nedlands, Australia.

Assessment of endocardial fibroelastosis by cardiac MRI

A 17-year-old patient was referred to the Unit of Cardiovascular Medicine at the Leeds General Infirmary (Leeds, United Kingdom) for a cardiac magnetic resonance imaging (MRI) scan. At the age of two months, she had developed dyspnea and was found to have unexplained left atrial enlargement. By the age of six years, she had developed significant tricuspid regurgitation and pulmonary hypertension. Cardiac catheterization at that time confirmed a mean pulmonary artery pressure of 50 mmHg, with no evidence of pulmonary artery stenosis or shunt. A diagnosis of restrictive cardiomyopathy secondary to primary endocardial fibroelastosis was established after right ventricular endomyocardial biopsy. Cardiac MRI using steady-state free precession cine imaging revealed the typical features of a restrictive cardiomyopathy (Figure 1A). The left ventricular cavity was small and showed normal systolic function. There was marked left atrial and right atrial dilation, global pericardial effusion, right ventricular hypertrophy and severe tricuspid regurgitation. The pulmonary arteries were dilated and the interventricular septum appeared flattened, consistent with right ventricular pressure overload (mean pulmonary artery pressure measured 80 mmHg at repeat cardiac catheterization at 17 years of age). In addition, the inferior vena cava was grossly dilated (Figure 1B) and a thrombus extended from the left atrial appendage into the left atrium (Figure 1C). Late gadolinium hyperenhancement imaging revealed a characteristic pattern of high-signal intensity confined to the endocardial surface of both ventricles and both atria (Figure 1D), representing fibrosis in these areas. Figure 1) A Four-chamber cine image demonstrating biatrial dilation, right ventricular hypertrophy, a small left ventricular cavity and pericardial effusion. B Cine image demonstrating dilation of the inferior vena cava (arrow). C Short-axis cine image demonstrating ... Cardiac MRI clearly demonstrates the pathognemonic features of endocardial fibroelastosis and can identify additional complications such as intracardiac thrombus.

Atrial enhancement by cardiovascular magnetic resonance in cardiac amyloidosis

A 78-year-old man was referred for cardiovascular magnetic resonance (CMR) to investigate symmetrical left ventricular hypertrophy identified by echocardiography. Electrocardiography showed first-degree heart block with a long duration (140 ms), low-voltage P wave, and atrial arrhythmia identified on 48 h Holter monitoring. CMR showed biatrial dilatation, severe left ventricular hypertrophy, and globally impaired …

The Role of Basic Leucine Zipper Protein‐Mediated Transcription in Physiological and Pathological Myocardial Hypertrophy

Accumulating evidence suggests that nuclear transcription factors from the basic leucine zipper (bZIP) family play an important role in cardiac development and function. This class includes the CREB/ATF family of transcription factors, namely CREB, cAMP response element modulator (CREM), ATF, and the related AP-1 and C/EBP families. An effort has been made to elucidate the role of specific bZIP members in the heart. Unfortunately, little insight could be gained from knockout experiments, either due to embryonic lethal phenotypes or functional compensation by other bZIP family members. Surprisingly, cardiac overexpression of several inhibitory transcription factors from the bZIP family, such as a nonphosphorylatable form of CREB (CREB(ser133)), a nonfunctional isoform of CREM, or ATF3 resulted in massive atrial dilatation. In order to try and characterize this pathway we have expressed the potent bZIP inhibitory protein, Jun dimerization protein 2 (JDP2), specifically in the mouse heart in a temporally controlled manner. Expression of JDP2 resulted in massive biatrial dilatation; loss of connexin 40 (Cx40), connexin43 (Cx43), and myosin light chain 2 (MLC2a) expression; atrioventricular defects in conduction; and a lethal phenotype. All these effects were independent of any developmental events acquired during adulthood, and were totally reversible upon abolishing the bZIP inhibition. The results of this article suggest that bZIP inhibition is sufficient to cause atrial dilation, that this dilatation is acquired postnatally, and that it is reversible upon the relief of inhibition. Thus, bZIP repressors may serve as novel drug targets for the prevention of atrial dilatation a major risk of atrial fibrillation (AF).