LA compression due to esophageal achalasia is uncommon. Apart from achalasia, four categories of abnormal structure could compress LA, which are defined by the origin and location: gastrointestinal structures, mediastinal structures, aorta and intracardial or pericardial structures, and pulmonary structures.1
LA compression can lead to low cardiac output, dyspnea, chest pain, pulmonary congestion, palpitation, and hemodynamic instability.1 Some patients with LA compression may develop heart failure.4,5 Comparing to congestive heart failure with LA enlargement, heart failure due to LA compression results from reduces LA volume and increases the refilling pressure. Accordingly, patients may also experience repeated syncope and arrhythmia.6,7 Upile et al. presented a case of achalasia with paroxysmal atrial fibrillation, whose heart rhythm reverted to sinus rhythm when food debris was removed from the esophagus.8 The mechanism for arrhythmia is not fully understood, but Volpi et al. proposed that the plausible explanation is mechanical stretching of the atrial myocardial fibers.9
The most common symptoms of esophageal achalasia are dysphagia and acid regurgitation.10 It is an uncommon esophageal motility disorder, with a prevalence of around 11 cases per 100,000 individuals.11 The pathophysiology of achalasia involves the degeneration of inhibitory neurons in the esophageal myenteric plexus but the exact cause remains unknown.12 Esophageal achalasia is often diagnosed using endoscopy. The finding of a bird beak shape of contrast pooling on the barium swallow esophagogram is typical.13 Clinically, esophageal manometry can be used to confirm the diagnosis.14 Current treatment mainly targeting the disruption of the lower esophageal sphincter, and no intervention had been verified to reverse the degeneration of esophageal neurons. Other treatment options include pneumatic dilatation, peroral endoscopic myotomy, botulinum toxin injection, and laparoscopic Heller myotomy with partial fundoplication. There is little evidence that calcium blockers or phosphodiesterase are effective, but these medications are commonly used in patients who cannot tolerate invasive interventions.14
TTE is the most common non-invasive diagnostic tool for extrinsic LA compression, and even a slight compression or deformity in the LA chamber can also be detected. Given a poor echocardiographic window, a transesophageal echocardiogram can be an alternative method to detect the anomaly. With the combination of TTE and carbonated beverage swallow tests, it is possible to differentiate gastrointestinal origins of extrinsic LA compression from the other mediastinal structures.15 Color Doppler echocardiography can also detect the turbulent flow in the LA and blood flow over the extrinsic mass. Contrast echocardiography can distinguish vascular or non-vascular structures that compress the LA.5 Further image tools such as computed tomography and magnetic resonance imaging, are usually performed to provide details of the features and origin of the mass.1 TTE can also be used to determine the effectiveness of treatment afterwards.16
This case report involves a patient with LA compression due to esophageal achalasia and gives a brief review of diagnoses and treatments of the disease. Extrinsic compression of the LA due to esophageal achalasia is rare, and the condition causes dysphagia that is concomitant with symptoms of heart failure. A prompt and accurate diagnosis is essential. TTE is a rapid, safe, and non-invasive diagnostic tool for LA compression, and a bird beak shape of contrast pooling on the barium swallow esophagogram is indicative of esophageal achalasia. Although the condition is relatively rare, the diagnosis must be considered in patients presenting with simultaneous dyspnea and dysphagia.