OpenHealth Q&A

Exploring Causes of Right to Left Transpulmonary Shunt Beyond PAVM

Introduction: - 20 years old, female - Diagnosed with Ehlers-Danlos Syndrome - Not currently taking any medications Hello, I've been grappling with certain health issues for the past two years, and despite consulting with various doctors, I’ve been unable to find any answers. I’m reaching out here in hopes of gaining some insight. For over two years, I’ve experienced significant shortness of breath when engaging in any form of activity. Additionally, I often experience intermittent drops in my oxygen levels, occurring several times daily. These drops are usually brief but occasionally last longer during sleep. This frequent fluctuation leads to feelings of fatigue and dizziness, accompanied by an unusual pressure sensation in my chest, neck, and head. It’s not the same as the feeling of needing air or tightness in my chest; it's more akin to the pressure felt during a Valsalva maneuver - something I’m not intentionally doing. Overall, it’s an uncomfortable experience. The inconsistency of these oxygen drop episodes is particularly perplexing. They can catch me off-guard while exercising (though shortness of breath happens every time), and they also occur during sleep. Unfortunately, because I can’t predict or replicate them at will, it’s difficult to demonstrate during short doctor visits, leading to skepticism about my experiences. Most of the time, my oxygen saturation remains stable, but when it dips, it can fall into the low 80s. I start feeling unwell around 91-92%, and the situation worsens as levels decrease; the lowest I’ve recorded was 76%, which nearly caused me to black out. Due to the unpredictability of these events, my doctors have initially attributed them to erroneous readings. Yet, I’m confident in my measurements: I avoid nail polish, ensure my hands are warm, and monitor the perfusion index. I’ve learned to use the pulse oximeter correctly and have verified that my device's readings align with those taken in medical offices, affirming its accuracy. After undergoing a standard chest X-ray, a bubble echocardiogram was performed to check for a patent foramen ovale (PFO), which was ruled out. Instead, the echocardiogram report indicated a delay in bubble appearance in the left atrium, suggesting a right to left/transpulmonary shunt. A subsequent chest CT scan aimed at identifying a possible pulmonary arteriovenous malformation (PAVM) yielded negative results. When the doctors encountered this mysterious shunt, they admitted their uncertainty about its source, unable to provide a diagnosis or treatment options. While I don’t believe these symptoms pose a severe threat to my life, I am exhausted from feeling unwell. Doctors have stated that without identifying the cause, they cannot offer relief or treatment, so I’m left to endure the episodes of oxygen drops and persistent shortness of breath with minimal exertion, including simple walking. They recommended diaphragmatic breathing during these episodes, but this technique hasn’t alleviated my symptoms at all; I simply have to endure it. I am seeking opinions on what types of shunts could exist outside of a PAVM, or any alternative explanations for my condition. Any insights would be greatly appreciated!

Answers (1)

2/14/2026, 5:51:00 AM

OpenHealth AI

It sounds like you're dealing with very challenging symptoms related to your Ehlers-Danlos Syndrome (EDS) and the identified right to left transpulmonary shunt. I understand how exhausting and frustrating this situation must be, especially with the unpredictability of your oxygen levels and the pressure sensations you describe. Here’s a breakdown of potential causes and practical next steps you can take.

Main Points:

1. Your symptoms and the identified transpulmonary shunt require further exploration beyond typical origins.
2. Ehlers-Danlos Syndrome may predispose you to conditions affecting the lungs and vascular system.
3. Immediate self-care strategies can help you manage symptoms while seeking further investigation.

Explanation:

The right to left transpulmonary shunt you've experienced, while not linked to a patent foramen ovale (PFO) or pulmonary arteriovenous malformation (PAVM), can arise from various conditions including:

• Pulmonary capillary dysplasia or other vascular abnormalities.
• Intrapulmonary shunts related to vascular changes due to EDS, which could affect how blood flows through the lungs and interacts with your oxygen levels.
• Ventilation-perfusion mismatch due to compromised lung function or blood flow dynamics in EDS.

The pressure sensations and shortness of breath you experience could stem from inadequate oxygen delivery to tissues, which is affected by your shunting. Your symptoms may be exacerbated by physical exertion, requiring your body to demand more oxygen than is available due to the irregular blood flow.

Specific Next Steps:

1. Document Symptoms: Keep a detailed diary of your symptoms, noting when oxygen dips occur, associated activities, and other relevant factors (stress, time of day, etc.). This may help healthcare professionals identify triggers or patterns.

2. Monitor Oxygen Levels: Continue to use your pulse oximeter accurately, especially during exertion or when experiencing symptoms. Record the context for each reading to build a comprehensive picture.

3. Breathing Techniques: Since diaphragmatic breathing isn't helping, consider exploring other techniques like pursed-lip breathing or gentle yoga exercises tailored for respiratory support. Apps or online classes may offer guided practices suited for this.

4. Hydration & Nutrition: Ensure you’re well-hydrated and consuming a balanced diet rich in vitamins and minerals. Dehydration can worsen your symptoms.

5. Specialist Consultation: Following the recommendations of your healthcare team, seek consultations with specialists who have experience with connective tissue disorders (such as a cardiologist or pulmonologist familiar with EDS) and inquire about advanced imaging studies like a right heart catheterization or more specific lung function tests that may reveal underlying issues.

6. Support Groups: Consider joining groups for individuals with EDS or related experiences. Sharing insights and challenges with others who understand your situation can provide both emotional support and practical tips.

Remember, while it can be disheartening not to have clear answers, the goal is to manage your symptoms effectively while the investigation continues. You deserve support in this journey—don’t hesitate to advocate for your health needs.