Cardiospasm: Causes, Symptoms, and Treatment

Esophageal achalasia (esophageal cardiospasm) is a rare neuromuscular disorder characterized by poor relaxation of the lower esophageal sphincter and weakened or absent distal esophageal peristalsis. This results in food retention, dysphagia, regurgitation, and chest pain. Without treatment, dilation and "congestion" of the esophagus develop, leading to a risk of aspiration and weight loss. Modern diagnostics rely on high-resolution manometry using the Chicago Classification criteria v4.0, as well as functional tests (FLIP, TBE). [1]

The main therapeutic goals are to reduce resistance at the esophagogastric junction, restore transit, and prevent complications. Effective first-line options include pneumatic dilation, laparoscopic Heller myotomy with partial fundoplication, and peroral endoscopic myotomy (POEM). For manometry type III (spastic variant), POEM demonstrates superiority, while for types I-II, the results of several methods are comparable. The choice of strategy depends on the achalasia subtype, age, patient preference, and center expertise. [2]

Even with successful sphincter decompression, some patients require long-term observation due to the risk of gastroesophageal reflux disease and esophagitis, especially after POEM. A small proportion of patients still experience pain due to visceral hypersensitivity, which requires additional non-motor strategies. [3]

Code according to ICD-10 and ICD-11

In the International Classification of Diseases, Tenth Revision, achalasia is coded as K22.0 "Achalasia of cardia." Synonyms include "cardiospasm" and "achalasia, unspecified." This code is used for all idiopathic forms of achalasia in adults (congenital forms are excluded and coded separately). [4]

The International Classification of Diseases, Eleventh Revision, uses DA21.0 "Achalasia" in the section "Esophageal Motility Disorders" (DA21). The description emphasizes two key features: loss of peristalsis in the distal esophagus and a relaxation defect of the lower esophageal sphincter; the term "cardiospasm" is also included in this section. [5]

Table 1. Correspondence of ICD codes for achalasia (cardiospasm)

Clinical situation	ICD-10	ICD-11
Achalasia (cardiospasm), adult forms	K22.0	DA21.0
Esophageal obstruction (for differentiation, pseudoachalasia)	K22.2	DA21.2/DA21.3 (by phenotype)
Congenital anomalies of the esophagus	Q39.x	LB12.x (congenital)

Epidemiology

Achalasia is a rare disease. Global estimates indicate an incidence of approximately 0.6-1.0 cases per 100,000 people per year and a prevalence of approximately 10 per 100,000; in some databases and insurance cohorts, rates are higher due to better detection. The disease occurs at any age, but is most common in middle-aged and elderly people. [6]

Population-based studies in recent years have documented an increasing trend in detection rates, which is attributed to the availability of high-resolution manometry and endoscopic therapy. However, significant geographic differences remain. [7]

The risk of esophageal cancer in patients with long-standing achalasia is increased compared to the general population (primarily squamous cell carcinoma), although the absolute annual incidence is low. This influences recommendations for surveillance and vigilance, but does not mean mandatory screening for everyone without risk factors. [8]

Table 2. Epidemiological landmarks

Indicator	Grade
Morbidity	0.6-1.0 per 100,000/year
Prevalence	~10 per 100,000
Trend	Increased detection rates (diagnostic improvements)
Risk of esophageal cancer	Elevated relative to the population; absolute risk is low on an annual basis

Reasons

Idiopathic achalasia is associated with the degeneration of inhibitory neurons (nitritergic) in the intermuscular plexus (Auerbach's plexus) of the distal esophagus and the lower esophageal sphincter. The result is a defect in its relaxation and loss of peristalsis. Autoimmune mechanisms and previous viral infections are discussed as etiologies, but a convincing causal link at the population level has not been proven. [9]

Rare secondary ("pseudo") achalasia is caused by tumors of the cardiac region of the stomach/esophageal-gastric junction or Chagas disease (in endemic regions). In these cases, conduction is impaired or the outlet is mechanically blocked, which manometrically can mimic achalasia. This is why organic causes are always excluded at the initial diagnosis. [10]

Genetic factors play a limited role; family observations and associations with some syndromes have been described, but this is rare in clinical practice. Concomitant conditions that influence the course of the disease (e.g., severe gastroesophageal reflux disease after interventions) are more significant. [11]

Risk factors

There are no reliably established modifiable risk factors for idiopathic achalasia. The disease can develop at any age, although it is most often diagnosed in people over 40–50 years of age. There are few gender differences in incidence. [12]

Red flags for secondary (pseudo) achalasia include rapid onset of symptoms (months), significant weight loss, age over 60 years, and a history of cancer. In such scenarios, enhanced cancer surveillance (endoscopy with biopsies and/or CT) is required. [13]

For oncological outcomes (over a period of decades), additional "marker" factors include prolonged dietary stagnation, candidal esophagitis, and male gender; absolute risks remain low on an annual basis. The decision to monitor is individualized. [14]

Table 3. What should alert a doctor to the possibility of "pseudo" achalasia

Sign	Why is it important?
Rapid progression of dysphagia (weeks-months)	Possible tumor obstruction
Severe weight loss	Oncological process
Age >60 years, history of cancer	Higher prior probability of cancer
Unusual endoscopic appearance/rigid cardia	Further biopsies/CT scans are required.

Pathogenesis

Damage to inhibitory neurons leads to increased basal tone and incomplete relaxation of the lower esophageal sphincter during pharynx. Simultaneously, loss of peristalsis disrupts bolus movement, causing wall distension and pain. In later stages, a "megaesophagus" develops with an S-shaped deformity and marked contrast retention on TBE. [15]

The Chicago Classification System v4.0 distinguishes three subtypes of achalasia based on manometry: type I – “classic” (total aperistalsis without pan-pressurization), type II – with pan-pressurization in at least 20% of swallows, type III – spastic (premature contractions without normal peristalsis). The subtype is important for prognosis and choice of therapy. [16]

Complementary methods include FLIP panometry (assessment of transition zone distensibility) and TBE (quantitative barium retention). Low distensibility on FLIP and a high barium column at 5 minutes support the diagnosis and help monitor the effect of treatment. [17]

Symptoms

The leading symptom is dysphagia for both solid and liquid foods. Patients describe a feeling of "getting stuck" behind the breastbone, the need to drink water, and to eat slowly and in small portions. Regurgitation of undigested food is common, especially at night, causing coughing and aspiration episodes. [18]

Substernal chest pain and a burning sensation are present in some patients, but are nonspecific and require exclusion of a cardiac cause in the acute onset. In later stages, weight loss, fatigue, and sleep disturbances may occur. In children, achalasia may present with food refusal, vomiting, and developmental delays. [19]

Symptoms are often aggravated by carbonated and very cold/hot drinks. Paradoxically, "soft" foods sometimes move more slowly than solid ones due to a weak peristaltic trigger. [20]

Classification, forms and stages

According to the Chicago Classification v4.0: type I - no peristalsis, no pan-pressurization; type II - no peristalsis, ≥20% pan-pressurization; type III - ≥20% premature contractions without adequate peristalsis, with defective sphincter relaxation (elevated IRP). These criteria are interpreted based on high-resolution manometry with mandatory verification of clinical relevance. [21]

A separate category is junctional outflow tract obstruction (EGJOO): elevated IRP with preserved peristalsis and ambiguous clinical findings. In v4.0, EGJOO requires confirmation by supportive tests (TBE/FLIP) and exclusion of structural obstruction. This is important to avoid "overtreating" patients with unnecessary myotomy. [22]

Based on the stage of morphological changes, a distinction is made between early (without significant dilation), advanced (dilation with delayed contrast), and advanced with esophageal deformation. Morphology influences the tactics (for example, the extent of myotomy and the expected frequency of repeat procedures). [23]

Table 4. Subtypes of achalasia according to the Chicago classification v4.0 (simplified)

Subtype	Manometric criterion	Clinical notes
Type I	Elevated IRP; 100% ineffective swallows without pan-pressurization	The answer to PD/LHM is good
Type II	Elevated IRP; ≥20% pan-pressurization	The best outcomes for all methods
Type III	Elevated IRP; ≥20% premature contractions	Extended myotomy (POEM) is preferred

Complications and consequences

Without treatment, esophageal dilation and deformation progresses, with severe food and fluid retention, leading to weight loss, micronutrient deficiencies, and repeated aspirations. This increases the risk of respiratory infections and hospitalizations. [24]

After effective decompression, the most common late sequelae are gastroesophageal reflux disease and erosive esophagitis, especially after POEM, requiring proton pump inhibitor prophylaxis and observation.[25]

The oncological risk (primarily squamous cell carcinoma) is increased in long-standing cases, but the absolute annual incidence is low; the issue of routine screening is decided individually, taking into account risk factors and the duration of the disease. [26]

Table 5. Common complications and how to prevent them

Complication	Prevention/tactics
Aspiration, pneumonia	Early decompression, sleeping with the head of the bed elevated
Nutritional deficiencies	Dietary support before and after intervention
Gastroesophageal reflux disease/esophagitis	PPI after myotomy, symptom control, endoscopy as indicated
Esophageal cancer (rare, long-term)	Individualized alertness, treatment of stasis

When to see a doctor

Immediately - in case of acute pressing pain behind the sternum with shortness of breath, cold sweat, radiating to the arm/jaw: first, it is necessary to exclude acute coronary syndrome according to cardiological algorithms, even if achalasia was previously diagnosed. [27]

Planned treatment is indicated for increasing dysphagia (for solid and/or liquid food), nocturnal regurgitation, weight loss, and coughing during meals or at night. Early referral for specialized diagnostics increases the chances of minimally invasive and effective treatment. [28]

After the treatment, if heartburn, chest pain, vomiting with food residue, or repeated pneumonia occur, a correction of therapy or a control assessment (endoscopy, pH-impedance analysis, TBE/FLIP) may be required. [29]

Diagnostics

Step 1. Rule out acute and structural causes. For acute pain, an electrocardiogram and high-sensitivity troponin are performed according to standard protocols. Esophagogastroduodenoscopy is then performed to rule out tumors of the esophageal-gastric junction, strictures, eosinophilic esophagitis, and other causes of dysphagia. If pseudoachalasia is suspected, CT/endosonography is additionally performed and biopsies are taken. [30]

Step 2. Confirm motility. High-resolution manometry is the "gold standard": it records an elevated integrated relaxation index (IRP) and the nature of peristaltic disturbances (types I-III). Interpretation should be consistent with the Chicago Classification v4.0, including maneuvers and assessment in different positions. [31]

Step 3. Supportive tests. Timed Barium Esophagram is a standardized X-ray examination: the patient rapidly drinks 150-200 ml of barium sulfate, with images taken at 1, 2, and 5 minutes; a high barium column height at 5 minutes supports the diagnosis and helps evaluate the effect of treatment. Modern studies suggest a threshold of ≥2 cm at 5 minutes as a convenient diagnostic guideline. [32]

Step 4. FLIP panometry. During endoscopy, a thin balloon probe measures the distensibility of the esophagogastric junction (EGJ-DI) and the pattern of wall responses to distension. Low distensibility indicates functional obstruction; normal distensibility has a high negative predictive value for EGJ-type disorders. FLIP is useful for borderline manometry results and for calibrating the myotomy volume. [33]

Table 6. Diagnostic methods and their role

Method	What does it show?	When it is especially useful
Endoscopy ± CT/biopsies	Exclusion of "pseudo"-achalasia, strictures, EoE	At the start for everyone
HRM (CC v4.0)	Confirmation of achalasia, types I-III	Choice of treatment method
TBE	Quantitative barium retention (1-5 min)	Diagnosis and monitoring of the effect
FLIP	EGJ extensibility, reactivity	Borderline cases, planning myotomy

Differential diagnosis

Pseudoachalasia (a tumor of the cardiac portion of the stomach/junction) is the main "double," especially with rapid progression and significant weight loss. Endoscopy with targeted biopsies and CT scanning resolve the issue. [34]

EGJOO (outflow obstruction): elevated IRP with partially preserved peristalsis; in CC v4.0, the diagnosis must be confirmed by TBE/FLIP and clinical examination. Incorrect interpretation leads to excessive myotomies. [35]

Other motility disorders: distal spasm, hypercontractile ("Jackhammer") esophagus - here, sphincter relaxation is normal, and the problem is the prematurity/strength of the waves; HRM and FLIP help. In cases of severe heartburn and strictures, severe gastroesophageal reflux disease is considered, and in cases of a rigid tube, systemic scleroderma. [36]

Treatment

The basic principle is to reduce resistance at the esophageal-gastric junction. Three first-line methods are pneumatic dilation (PD), laparoscopic Heller myotomy with partial fundoplication (LHM), and peroral endoscopic myotomy (POEM). For types I-II achalasia, PD, LHM, and POEM provide comparable clinical efficacy in experienced teams; the choice depends on age, preference, and local expertise. For type III (spastic), POEM is preferred due to the ability to perform a long myotomy along the esophageal body. [37]

Pneumodilation is a balloon-assisted dilation of the sphincter with inflation at a fixed pressure under X-ray guidance. This method is most effective in types I-II and in older patients; a series of dilations is often required for a lasting result. Serious complications are rare, but perforation is possible, so the procedure should be performed in experienced centers. In the long term, PD is superior to botulinum toxin and is often as good as myotomy in selected patients. [38]

Laparoscopic Heller myotomy with partial fundoplication (usually Dor) provides reliable and durable pressure gradient reduction with reflux protection. Advantages include a low reflux rate compared with POEM and a large historical data set. Limitations include invasiveness, the need for general anesthesia, and the risk of complications typical of laparoscopy. Efficacy is high in types I-II; in type III, undercutting is possible unless an extended superior myotomy is performed. [39]

POEM is endoscopic submucosal tunneling and dissection of the longitudinal and circular sphincter fibers and distal esophagus. This method offers high clinical efficacy (according to series and reviews, ~90% at 6-12 months) and is particularly useful in type III (possibility of long myotomy). The main disadvantage is a higher incidence of postprocedural gastroesophageal reflux disease, which requires proton pump inhibitor prophylaxis and observation. [40]

Pharmacotherapy (short-acting nitrates, calcium channel blockers) is used in patients unsuitable for invasive procedures or as a "bridge" to radical procedures. The symptomatic effect is often short-lived and limited by side effects (headache, hypotension), so these agents are not considered a long-term solution. Botulinum toxin provides temporary relief lasting months and is indicated for fragile patients for whom dilation/myotomy is contraindicated. [41]

Choice of method based on subtype. For types I-II, PD, LCM, or POEM are acceptable with comparable outcomes. The choice is discussed with the patient, taking into account age, reflux risk, availability of repeat procedures, and preferences. For type III, an extended myotomy is appropriate—usually POEM (up to 15-20 cm along the esophageal body) or extended LCM, with appropriate expertise. This "subtype ↔ method" correspondence improves long-term outcomes and reduces the need for retherapy. [42]

Personalization with FLIP/TBE. FLIP allows for real-time assessment of the transition zone distensibility and fine-tuning of myotomy length/depth; after the procedure, the target increase in the distensibility index correlates with symptom relief. TBE quantitatively confirms improved voiding (barium column reduction at 5 minutes) and is convenient for dynamic monitoring in outpatient practice. This "dual" objective monitoring reduces variability in success assessment. [43]

Prevention and treatment of reflux after myotomy is essential. After POEM, most patients are prescribed full-dose proton pump inhibitors with subsequent titration; in case of symptomatic recurrence, pH-impedance monitoring and therapy adjustments are performed. After LCM, partial fundoplication reduces the incidence of reflux, but monitoring is still required. In refractory esophagitis, increased antisecretory therapy, positional measures, and weight control are discussed. [44]

Retherapy and complications. In case of incomplete response after PD, repeated dilations of increased diameter are possible; after LCM and POEM, recurrence may be associated with submyotomy or scarring – further exploration (HRM, FLIP, TBE) and targeted repeat myotomy or an alternative technique are indicated. Complications of invasive methods are rare and manageable in experienced centers; perforations are more often associated with PD, and clinically significant gastroesophageal reflux disease – with POEM. [45]

Comorbidities and quality of life. Before and after treatment, it is important to eat small meals, chew thoroughly, sleep with the head of the bed elevated, and avoid heavy late dinners and carbonated beverages. Patient-oriented goals include returning to a normal diet, reducing nocturnal regurgitation and anxiety, and normalizing weight; "perfect" manometry is not an end in itself. If pain persists despite adequate decompression, pain neuromodulation strategies are helpful. [46]

Table 7. Comparison of the main treatment methods (summary)

Method	Strengths	Limitations/Risks	Who is it suitable for?
Pneumodilation	Fast, minimally invasive; good for the elderly	Risk of perforation; series of procedures may be necessary	Types I-II, preference for endoscopy
LHM + partial fundoplication	Long lasting; lower reflux rate	Invasiveness	Types I-II; young, with availability of a surgeon
POEM	High efficiency; long myotomy	More frequent gastroesophageal reflux disease	Type III; relapse after other methods
Botulinum toxin	Suitable for fragile patients	Short-term effect	When PD/myotomy is not possible

Prevention

There is no primary prevention for achalasia. However, complications can be prevented by sleeping with the head of the bed elevated, avoiding heavy late dinners and carbonated drinks, chewing food thoroughly, and drinking small sips of warm liquid. These techniques reduce the likelihood of nocturnal regurgitation and aspiration until definitive treatment is performed. [47]

After myotomy, reflux prevention is important: antisecretory therapy as indicated, weight control, and positional measures. Patients with persistent TBE delay and low FLIP compliance may require retherapy, so follow-up studies are scheduled in advance. [48]

Patients with a long history of stasis and cancer risk factors are advised of the "alarm signs" and the advisability of individualized monitoring. A conscious choice of a center with experience in PD/LHM/POEM reduces the risk of complications and reinterventions. [49]

Forecast

With proper selection and implementation of the intervention (PD, LCM, or POEM), most patients achieve sustained relief of dysphagia and regurgitation. For type III, a longer myotomy (usually POEM) provides a better clinical effect than short myotomies. For types I-II, the differences between methods are smaller; the reflux risk profile and patient preference come first. [50]

Long-term quality of life is high when reflux is controlled and retherapy is promptly instituted when symptoms recur. Absolute annual cancer risks are low, but elevated relative to the general population in very long-standing cases—this argues for eliminating stasis and promoting informed awareness rather than constant invasive screening for everyone. [51]

Table 8. Predictive factors and practical conclusions

Factor	Influence	What to do
Achalasia subtype (I-III)	Determines the choice of method	Type III → extended myotomy (usually POEM)
Age/comorbidity	Risks of anesthesia and surgery	Consider PD/botulinum toxin
Reflux after treatment	Esophagitis, decreased quality of life	STI prevention, monitoring
Maintaining stasis on TBE/low EGJ-DI	Risk of relapse	FLIP/TBE control, retherapy

FAQ - Frequently Asked Questions

Can cardiospasm be "cured permanently"? The goal is permanent relief of the obstruction and normal eating. For many, the effects of PD/LHM/POEM last for years; sometimes retherapy or a change in method is necessary, which is normal for a chronic motor disorder. [52]

Which method is better: PD, LHM, or POEM? For types I-II, the results are comparable when performed well; for type III, a long myotomy (usually POEM) is preferred. The choice is discussed taking into account age, reflux, and the experience of the center. [53]

Is it true that almost everyone will experience reflux after POEM? The risk is higher than after LHM with fundoplication, but it is manageable with medications and regimen; some patients experience no heartburn at all. A follow-up plan is needed. [54]

Are there "magic pills" instead of procedures? Medications (nitrates, calcium channel blockers) provide short-term relief and are not suitable for everyone; botulinum toxin is a temporary option for "fragile" patients. PD, LHM, or POEM provide a more radical solution. [55]

How can you tell if the treatment has helped? By how you feel (Eckard scale), and by objective tests: TBE (decrease in barium level at 5 minutes) and/or FLIP (increase in transition zone extensibility). This increases confidence and helps you adjust your strategy in a timely manner. [56]