1School of Medicine, Queen’s University, Kingston, ON, Canada;
2Department of Medicine, Queen’s University, Kingston, ON, Canada
Dysphagia megalatriensis is a rare cause of esophageal dysphagia caused by left atrial enlargement (LAE). LAE is often caused by chronic atrial fibrillation, chronic hypertension and left ventricular dysfunction, or mitral valvulopathy. Here, we report a case of an 87-year-old female presenting with a 4-week history of progressive dysphagia and odynophagia to solid foods and a 2-week history to liquids. The patient had intermittent nausea and vomiting for the past 6 months, accompanied by an unintentional weight loss of 40 lbs over the same time period. This had worsened since the onset of the chest pain and she now experienced daily vomiting. Her past medical history was significant for hypertension, coronary artery disease, atrial fibrillation (AF) requiring AV node ablation with pacemaker insertion, and COPD. Echocardiography revealed a severely enlarged left atrium with patent foramen ovale, mitral annular calcification, severe TR and an elevated pulmonary artery systolic pressure of 77 mmHg. This case highlights the importance of dysphagia megalatriensis as a pertinent differential to consider in patients with long-standing cardiac conditions presenting with dysphagia and the importance of utilizing the Canadian Association of Gastroenterology’s diagnostic approach to uninvestigated dysphagia to come to the appropriate diagnosis. Additionally, we discuss the merits of rate vs. rhythm control in patients with AF-induced LAE. While there is some data to suggest that rhythm control is associated with poor outcomes in patients with normal to mild LAE, there is limited information is available on patients with existing LAE who would likely benefit from rhythm control.
La « dysphagia megalatriensis » est une cause rare de dysphagie œsophagienne provoquée par une hypertrophie auriculaire gauche (HAG). Cette hypertrophie est souvent causée par une fibrillation auriculaire chronique, une hypertension chronique et un dysfonctionnement du ventricule gauche ou une valvulopathie mitrale. Nous rapportons ici le cas d’une femme de 87 ans présentant une dysphagie et une odynophagie progressives aux aliments solides depuis quatre semaines et aux liquides depuis deux semaines. Au cours des six derniers mois, la patiente a connu des nausées et vomissements intermittents, accompagnés d’une perte de poids involontaire de 18 kg. Cette situation s’est aggravée depuis l’apparition d’une douleur thoracique, et elle vomit maintenant tous les jours. Elle présente certains antécédents médicaux d’importance : hypertension, coronaropathie, fibrillation auriculaire nécessitant l’ablation du nœud auriculoventriculaire et la mise en place d’un stimulateur cardiaque et MPOC. L’échocardiographie révèle une hypertrophie grave de l’atrium gauche accompagnée d’un foramen ovale perméable, une calcification annulaire mitrale, une régurgitation tricuspide grave et une pression artérielle pulmonaire systolique élevée de 77 mmHg. Ce cas met en évidence l’importance de la « dysphagia megalatriensis » comme diagnostic différentiel pertinent à considérer chez les patients atteints depuis longtemps de problèmes cardiaques et présentant une dysphagie ainsi que l’importance d’utiliser la démarche diagnostique pour les dysphagies non explorées de l’Association canadienne de gastroentérologie pour poser le bon diagnostic. Par ailleurs, nous abordons les avantages d’une maîtrise de la fréquence par rapport à celle du rythme cardiaque chez les patients atteints d’une HAG induite par la fibrillation auriculaire. Bien que certaines données laissent entendre que la maîtrise du rythme est associée à de mauvais résultats chez les patients atteints d’une HAG normale ou légère, on dispose de peu de renseignements sur les patients présentant une HAG qui bénéficieraient probablement d’une maîtrise du rythme cardiaque.
Key words: dysphagia megalatriensis, left atrial enlargement
Corresponding Author: Don Thiwanka Wijeratne: firstname.lastname@example.org
Submitted: 7 December 2020; Accepted: 1 February 2021; Published: 15 December 2021
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This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 International (CC BY-NC 4.0)(https://creativecommons.org/licenses/by-nc/4.0/).
Dysphagia megalatriensis is an uncommon and serious cause of dysphagia, typically presenting in patients with longstanding history of cardiac conditions (e.g., atrial fibrillation, hypertension).
The diagnosis can be confirmed by computed tomography and echocardiography after other life-threatening conditions have been excluded.
Management must be focused on the underlying cause(s) of left atrial enlargement, but guideline-based treatment options are lacking.
An 87-year-old female presented with a 4-week history of progressive dysphagia and odynophagia to solid foods and a 2-week history to liquids. Her symptoms were reproducible with 3–4 boluses of food over her central chest. A trial of antacids and pantoprazole for gastroesophageal reflux disease yielded no improvement. Her past medical history was significant for hypertension, coronary artery disease, and atrial fibrillation (AF) requiring AV node ablation with pacemaker insertion, and COPD. Her medications included apixaban, furosemide, atenolol, hydrochlorothiazide, and nitroglycerin patch. The patient had intermittent nausea and vomiting for the past 6 months, accompanied by an unintentional weight loss of 40 lbs over the same time period. This had worsened since the onset of the chest pain and she now experienced vomiting daily.
On examination, vital signs were normal (BP: 130/80; HR: 78) and the patient was afebrile. Severe jugular vein distention was present with a prominent CV wave. There was an III/VI in intensity holosystolic murmur best heard at the right mid-sternal border and an II/VI systolic murmur best heard over the apex. In addition, elevated jugular venous pressure and two pansystolic murmurs were present on cardiovascular examination, which were later confirmed to be severe tricuspid regurgitation (TR) and mild mitral regurgitation. Respiratory exam was normal with clear lungs. Slight, nontender, bilateral nonpitting edema to the mid-calf was present. The abdomen was soft with slight epigastric tenderness on palpation.
Chest x-ray showed an enlarged cardiac silhouette. Brain natriuretic peptide concentration was 598 ng/L. Troponins and echocardiograph were negative for ischemic changes. A CT of the chest, abdomen, and pelvis revealed massive distension of the left atrium causing severe mass effect and compression onto the mid-esophagus with associated obstruction and dilation of the proximal esophagus (Figure 1). This was thought to be the likely cause of the patient’s dysphagia and chest discomfort leading to weight loss. A previous echocardiography conducted in 2003 showed moderate bi-atrial enlargement, but rhythm control therapy for her AF was never initiated. Repeat echocardiography on this admission confirmed severely enlarged left atrium (LA diameter of 4.5 cm; LA volume index of 106.0 mL/m2) (Figure 2) with patent foramen ovale (PFO), mitral annular calcification, severe TR, left ventricular diastolic dysfunction, and an elevated pulmonary artery systolic pressure of 77 mmHg. There was no aortic valvulopathy noted. Upper endoscopy revealed circumferential, ulcerated, necrotic lesions, which showed active esophagitis and necrosis without signs of malignancy on biopsy.
Figure 1. A chest computed tomography (CT) in an 87-year-old woman with dysphagia megalatriensis. Results indicate severe compression of the esophagus (arrow).
Note: LA = left atrium.
Figure 2. Transthoracic echocardiography apical 2 chamber view showing severe left atrial enlargement.
Note: LA = left atrium; LV = left ventricle.
Unfortunately, no curative treatment was possible, and the patient denied any further interventions. Therapy was directed at symptom management. Her severe left atrial enlargement (LAE) and chest pain were managed with metoprolol and a nitroglycerine patch, which proved to be ineffective. She subsequently requested and qualified for MAID.
Dysphagia, or difficulty swallowing, is a common clinical presentation, the prevalence of which is increasing in the aging population in Canada. It can be broadly categorized into oropharyngeal dysphasia and esophageal dysphagia. Oropharyngeal dysphagia is characterized by difficulty initiating a swallow and may be accompanied by aspiration, nasopharyngeal regurgitation, and a sensation of residual food remaining in the pharynx. Common causes of oropharyngeal dysphagia include stroke, Parkinson’s disease, peripheral neuropathy, and polymyositis. In contrast, esophageal dysphagia is caused by abnormal peristaltic motility or obstruction of a food bolus through the esophagus. Achalasia and scleroderma are common motility disorders, while carcinomas, strictures, and esophageal rings and webs are common obstructive causes of esophageal dysphagia.
An uncommon and often unrecognized cause of esophageal dysphagia is LAE. LAE is most commonly the result of conditions caused by pressure and/or volume overload. The left atrium is the most posterior cardiac chamber directly anterior to the esophagus. Massive LAE can cause dysphagia megalatriensis from extrinsic compression of the esophagus. Dysphagia megalatriensis was first described by Le Roux and Williams in 1969, where they reported a case of dysphagia from LAE in a 26-year-old woman with AF.1 Since then, relatively few cases of dysphagia megalatriensis have been reported. On reviewing the literature, Gajanana et al. previously reported a case of giant left atrium-induced dysphagia in a 65-year-old woman with AF.2 Similarly, Alhajiri et al. described the case of a 62-year-old woman with a history of AF, paroxysmal atrial tachycardia, and heart failure (HF) presenting with progressive dysphagia for solid foods and significant weight loss.3
It is difficult to ascertain the causative sequence of the patient’s LAE. Mitral valve disease and left ventricular dysfunction may result in pressure and/or volume overload, which, if left untreated, may lead to LA remodeling and LAE. Similarly, chronic hypertension can induce LAE. Although less common, isolated AF is also a cause of LAE. In a study by Sanfilippo et al., patients with persistent AF experienced a mean LA volume increase over time from 45 to 64 cm3.4 This remodeling can also occur in paroxysmal AF and is independent of loading conditions in the LA and left ventricular size or function.4 In another study, restoration and maintenance of sinus rhythm decreased atrial volumes.5 It is therefore reasonable to believe that this patient’s LAE was caused by a combination of persistent, chronic AF, hypertension, and left ventricular diastolic dysfunction.
This case highlights the challenge for clinicians in recognizing and diagnosing an uncommon cause of dysphagia. The patient’s case contained several unique features that warranted further exploration. Patients over the age of 50 years with new onset dysphagia and an associated alarm feature, such as unexplained weight loss in this patient, have a significantly increased risk of esophageal cancer. Although this patient’s upper endoscopy revealed necrotic lesions with active esophagitis in the mid-distal esophagus, no malignancy was noted on pathology. We therefore did not believe that this was the cause of her dysphagia and proceeded with a subsequent CT scan, which showed severe LAE with compression of the esophagus. Despite these clinical challenges, this patient’s rare cause of dysphagia highlights the strength of the CAG’s algorithm. We strongly suggest that clinicians utilize this algorithm in patients presenting with dysphagia.
As suggested by the Canadian Association of Gastroenterology (CAG),6 once oropharyngeal dysphagia is ruled out, patients with symptoms of esophageal dysphagia must be assessed by history and physical examination. A thorough history can distinguish between structural and motility disorders of the esophagus and highlight alarm features, including unexplained weight loss, vomiting, anemia, abdominal mass, gastrointestinal bleeding, and age greater than 50 years (Figure 3). The presence of an alarm feature could suggest structural and inflammatory lesions and merit referral for upper endoscopy. In the absence of cancer, further imaging studies, such as computerized tomography (CT) scan, are warranted.
Figure 3. The Canadian Association of Gastroenterology’s suggested diagnostic approach to uninvestigated dysphagia. *Imaging studies could include computed tomography (CT) and ultrasound. †Expertise (e.g., speech language pathologist or occupational therapist) and assessments (e.g., videofluoroscopic swallowing study [VFSS] or fiberoptic endoscopic evaluation of swallowing [FEES]) vary depending on regional availability.
Currently, there are no guideline-based treatment options available for LAE. Medical therapy is not available to reverse LA remodeling. Instead, the focus of care should be aimed at identifying and treating the underlying cardiac cause of LAE. Although rate and rhythm-control strategies improve symptoms, there is no significant difference in mortality nor quality of life between the two strategies in most patient groups.7 A rate control strategy is preferable in patients with the following factors: persistent AF, less symptomatic, age ≥ 65 years, no history of HF, and hypertension.7 Generally, a rhythm control strategy is recommended in patients with paroxysmal AF, newly diagnosed AF, age < 65 years, history of congestive HF exacerbated by AF, hypertension, and those who remain symptomatic despite optimization of rate control.7 In a study using the AFFIRM trial database, rhythm control, but not rate control, was associated with an increased risk in mortality and hospitalization in patients with LAE.8 However, limited information is available on patients with existing LAE who would likely benefit from rhythm control. This is an important consideration in patients with progressive and documented LAE to be considered for rhythm control beyond the usual indication for rhythm control for symptomatic AF. Furthermore, there is evidence to suggest that cardioversion9 and catheter ablation5 in AF patients can regress LA size by reverting the heart to sinus rhythm. Therefore, cardioversion and catheter ablation should be considered for patients with AF-induced LAE.
Our patient’s presentation is common, but her diagnosis was rare. Dysphagia megalatrienis is a pertinent differential consideration for patients with swallowing difficulties accompanied by a history of cardiac conditions, including, but not limited to, chronic AF, hypertension, left ventricular dysfunction, and mitral valvulopathy. Effective treatment must target the underlying cause of LAE. We highlight the importance of updating current recommendations regarding rhythm control in patients with documented and progressing LAE.
The authors received written consent from the patient prior to the submission of this clinical case report. Both MM and DTW contributed to the conception and design, and drafting and critical review of the original manuscript. MP contributed to the drafting of the original manuscript, and ZJ contributed to the critical review of the original manuscript. No funding was received for this clinical case report. Furthermore, all authors declare no conflicts of interest and take responsibility for the integrity of the content of the report.
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