Our Summary

This study looked back at the surgical treatment of a fairly uncommon heart condition called Peripheral Pulmonary Artery Stenosis (PPAS). This condition, often associated with Williams syndrome, Alagille syndrome, and elastin arteriopathy, is characterized by narrowing of almost all the lung’s artery openings. This leads to high pressure in the right side of the heart.

The researchers reviewed 145 cases of patients who had surgery to fix PPAS. These patients had a range of diagnoses, including Williams syndrome, Alagille syndrome, and elastin arteriopathy, among others.

Before surgery, the average pressure ratio between the right side of the heart and the main artery was 1.01. After surgery, this ratio dropped to 0.30, indicating a significant reduction in pressure in the right ventricle. On average, surgeons had to repair 17 artery openings during surgery, and the average length of the heart-lung bypass machine use was 398 minutes.

There were three deaths in the hospital (2.1% of patients) and four more deaths after discharge (2.9% of patients). 82 patients had follow-up tests, and 74 of these patients maintained a low pressure ratio.

The results suggest that surgery is highly effective in treating PPAS, leading to a 70% drop in right heart pressure. Three years after surgery, 94% of patients were still alive, and 90% of those tested still had low pressures.

FAQs

  1. What is Peripheral Pulmonary Artery Stenosis (PPAS) and what conditions is it often associated with?
  2. What was the outcome of the surgeries performed on patients with PPAS in terms of pressure reduction and survival rate?
  3. How many artery openings were typically repaired during the surgery and what was the average length of heart-lung bypass machine use?

Doctor’s Tip

A helpful tip a doctor might tell a patient about peripheral artery bypass is to follow post-operative care instructions carefully, including taking prescribed medications, attending follow-up appointments, and engaging in regular physical activity as recommended by the healthcare team. It is important to maintain a healthy lifestyle to support the success of the surgery and prevent further complications. It is also important to report any concerning symptoms or changes in health to your healthcare provider promptly.

Suitable For

Patients who are typically recommended for peripheral artery bypass surgery include those with severe narrowing or blockage of the arteries in the legs, arms, or other peripheral areas of the body. This can be due to conditions such as peripheral artery disease (PAD), atherosclerosis, or other vascular diseases. Patients may experience symptoms such as leg pain, cramping, numbness, or weakness, especially when walking or exercising.

Additionally, patients with conditions such as PPAS, as described in the study above, may also be recommended for peripheral artery bypass surgery. These patients may have narrowing or blockage of the pulmonary arteries, leading to high pressure in the right side of the heart and potentially life-threatening complications.

Overall, patients who are experiencing symptoms due to severe peripheral artery disease or other vascular conditions that are impacting their quality of life or putting them at risk for serious complications may be recommended for peripheral artery bypass surgery. It is important for patients to discuss their symptoms and treatment options with their healthcare provider to determine the best course of action for their individual situation.

Timeline

In summary, before peripheral artery bypass surgery, patients with PPAS experience high pressure in the right side of the heart due to narrowing of the lung’s artery openings. After surgery, there is a significant reduction in pressure in the right ventricle, with surgeons typically repairing 17 artery openings during the procedure. The average length of time on the heart-lung bypass machine is 398 minutes.

Following surgery, there were a total of seven deaths (4.9% of patients) either in the hospital or after discharge. However, the majority of patients who had follow-up tests maintained a low pressure ratio, indicating the effectiveness of the surgery in treating PPAS. Three years post-surgery, 94% of patients were still alive, with 90% maintaining low pressures.

What to Ask Your Doctor

  1. What is the success rate of peripheral artery bypass surgery in treating PPAS?

  2. How many artery openings will need to be repaired during the surgery?

  3. How long will I need to be on a heart-lung bypass machine during the surgery?

  4. What are the potential risks and complications associated with peripheral artery bypass surgery for PPAS?

  5. What is the expected recovery time after surgery?

  6. How often will follow-up tests be needed to monitor the effectiveness of the surgery?

  7. What lifestyle changes or medications will be necessary after surgery to maintain low heart pressure?

  8. What is the long-term prognosis for patients who have undergone peripheral artery bypass surgery for PPAS?

  9. Are there any alternative treatment options available for PPAS?

  10. How experienced is the surgical team in performing peripheral artery bypass surgery for PPAS?

Reference

Authors: Felmly LM, Mainwaring RD, Collins RT, Lechich K, Martin E, Ma M, Hanley FL. Journal: J Thorac Cardiovasc Surg. 2023 Apr;165(4):1493-1502.e2. doi: 10.1016/j.jtcvs.2022.07.037. Epub 2022 Aug 10. PMID: 36088147