Atrial Septostomy: Types, Symptoms, Causes, Diagnosis & Treatment

The heart has four chambers: two upper chambers called atria and two lower chambers called ventricles. Normally, the atria are separated by a wall called the septum. Atrial septostomy is a procedure that creates or enlarges a hole in this wall. 

This opening allows some blood from the oxygen-rich left atrium to mix with the oxygen-poor blood in the right atrium. This improves blood flow to the lungs, increasing oxygen delivery to the body and alleviating cyanosis. Atrial Septostomy is usually done for cyanotic congenital heart defects.

Atrial septostomy procedures can be categorised based on the technique used. Here are the two main types:

1. Rashkind Balloon Septostomy (Rashkind procedure): This minimally invasive approach is the most common type of atrial septostomy. A thin catheter with a balloon at its tip is inserted through a vein in the groyne and navigated to the heart. Once positioned across the atrial septum, the balloon is inflated to enlarge a naturally occurring opening called the foramen ovale. This opening usually closes shortly after birth, but the balloon septostomy creates a new passage for blood flow.
2. Surgical Septostomy: In some cases, a traditional surgical approach might be necessary. This open-heart surgery involves making an incision in the chest to access the heart directly. The surgeon then creates a controlled opening in the atrial septum using specialised instruments. This method offers more precise control over the size and location of the opening compared to the balloon technique.

Cyanotic congenital heart defects (CHDs) disrupt blood flow, leading to a deficiency of oxygenated blood reaching the body's tissues. This lack of oxygen manifests through various symptoms, some appearing immediately after birth and others developing over time. Here are the key signs to watch for:

1. Cyanosis:

• This is the hallmark symptom of cyanotic CHDs, characterised by a bluish or purplish discoloration of the skin, especially in areas rich in capillaries like the lips, fingertips, toes, and around the eyes.
• The severity of cyanosis can vary depending on the specific heart defect and the degree of oxygen deprivation. Sometimes, cyanosis might only be noticeable during feeding or crying spells when the baby exerts extra effort.

2. Respiratory Difficulties:

• Infants with cyanotic CHDs often experience rapid breathing (tachypnea) as their body struggles to compensate for the low oxygen levels.
• This rapid breathing can be shallow and laboured, causing distress and difficulty feeding.
• In some cases, babies might adopt a characteristic squatting position to ease their breathing discomfort.

3. Feeding Problems and Poor Weight Gain:

• The combination of low oxygen levels and breathing difficulties can significantly impact an infant's ability to feed effectively.
• They might tire easily while nursing or bottle-feeding, leading to poor weight gain and inadequate growth.
• Excessive sweating during feeding can also be a sign of exertion due to breathing difficulties.

4. Reduced Activity and Lethargy:

• Due to the lack of oxygen reaching their muscles and vital organs, babies with cyanotic CHDs often exhibit decreased activity levels and lethargy.
• They might appear less interested in playing or interacting with their surroundings and prefer to sleep excessively.

While the exact cause of cyanotic congenital heart defects (CHDs) remains a mystery in many cases, there are several factors that are believed to contribute to their development. Here are some of the potential causes:

1. Genetic Predisposition

• Family History: Having a close family member with a CHD increases your baby's risk.
• Gene Mutations: Specific gene mutations can disrupt the normal development of the heart during pregnancy. Some syndromes like Down syndrome, trisomy 13, Turner syndrome, Marfan syndrome, and Noonan syndrome are associated with a higher risk of CHDs.

2. Environmental Factors During Pregnancy

• Maternal Illnesses: Certain infections like rubella during pregnancy can disrupt foetal development, including heart formation.
• Poorly Controlled Blood Sugar: Uncontrolled diabetes in pregnant women can increase the risk of CHDs in their babies.
• Certain Medications and Teratogens: Some medications or recreational drugs taken during pregnancy can have harmful effects on the developing heart. It's crucial for pregnant women to consult their doctor before taking any medication, including over-the-counter drugs.
• Chemical Exposure: Exposure to certain environmental toxins or chemicals during pregnancy might also be a risk factor.

3. Problems with Blood Flow and Development

• Abnormal Septum Development: The septum, the wall separating the heart chambers, might not form correctly, leading to abnormal blood flow patterns. Atrial Septal Defects (ASD) are a type of septal abnormality that can contribute to cyanosis.
• Heart Valve Defects: Malformations of the heart valves can disrupt blood flow direction, causing some blood to flow backwards and mix oxygen-poor and oxygen-rich blood, leading to cyanosis.
• Abnormal Vessel Formation: Problems with the development of the major blood vessels leading to or from the heart can also contribute to cyanotic CHDs.

Cyanotic congenital heart defects (CCHDs) are a group of birth defects that affect the heart's structure, causing insufficient oxygenated blood to reach the body. While the exact causes of CCHDs remain under investigation, several factors can increase the risk of a baby developing this condition.

Genetics and Chromosomal Abnormalities:

• Family history of congenital heart defects: If a close family member has a heart defect, particularly a cyanotic one, the risk for a baby increases.
• Chromosomal abnormalities: Conditions like Down syndrome, trisomy 13, and Turner syndrome are linked to a higher risk of CCHDs. Genetic counseling can assess potential risks before pregnancy.

Maternal Health During Pregnancy:

• Certain illnesses: Rubella infection during pregnancy can significantly increase the risk of CCHDs. Maintaining up-to-date vaccinations, especially against rubella, is crucial.
• Uncontrolled diabetes: Poorly managed blood sugar in mothers with diabetes can contribute to CCHD development in the fetus.
• Certain medications: Some medications, particularly those not recommended during pregnancy, can have unintended consequences. Consulting a doctor before taking any medication while pregnant is essential.
• Substance abuse: Smoking, alcohol consumption, and illegal drug use during pregnancy can all increase the risk of CCHDs.

Other Risk Factors:

• Consanguinity: When parents are close relatives, the risk of passing on certain genetic conditions, including CCHDs, is higher.
• Maternal obesity: While the exact link is unclear, some studies suggest a possible association between maternal obesity and CCHDs.
• Low birth weight: Babies born with low birth weight are more susceptible to congenital heart defects, including cyanotic ones.

While completely preventing cyanotic congenital heart defects (CHDs) isn't possible, there are steps expecting parents can take to minimise the risk and promote a healthy pregnancy:

1. Preconception Counseling:

• Family history: If you or your partner has a family history of CHDs, consider preconception counselling with a genetic counsellor. They can assess the risk of your baby inheriting a genetic condition associated with CHDs.

2. Prenatal Care:

• Early and regular prenatal care: This is crucial for monitoring foetal development and detecting any potential abnormalities early on. Your doctor can recommend the appropriate schedule of prenatal visits and screenings.
• Folic acid supplementation: Taking 400 micrograms of folic acid daily, ideally starting at least three months before conception and continuing throughout the first trimester, can significantly reduce the risk of neural tube defects, which are sometimes associated with CHDs.
• Healthy diet: A balanced diet rich in fruits, vegetables, and whole grains provides essential nutrients for optimal foetal development.
• Maintaining a healthy weight: Obesity during pregnancy can increase the risk of various complications, including CHDs. Aim for a healthy weight before conception and maintain a healthy weight gain throughout pregnancy as recommended by your doctor.

3. Avoiding Teratogens:

• Infections: Certain infections during pregnancy, like rubella (German measles), cytomegalovirus (CMV), and toxoplasmosis, can increase the risk of CHDs. Maintaining good hygiene, receiving recommended vaccinations before pregnancy (like rubella), and avoiding contact with potential sources of these infections can help minimise risk.
• Smoking and secondhand smoke: Smoking significantly increases the risk of various pregnancy complications, including CHDs. Quitting smoking before conception and avoiding secondhand smoke exposure is essential.
• Alcohol and recreational drugs: Alcohol and recreational drugs can disrupt foetal development and increase the risk of birth defects. Abstaining from these substances throughout pregnancy is crucial.

4. Environmental Toxins:

• Occupational hazards : If your work environment involves exposure to certain chemicals or toxins, discuss potential risks with your doctor and explore ways to minimise exposure during pregnancy.
• Pollution: While it's difficult to completely control environmental exposure, minimising exposure to air and water pollution whenever possible can be beneficial.

5. Prenatal Screening and Testing:

• Prenatal ultrasounds: These routine scans can sometimes detect structural abnormalities in the foetal heart.
• Amniocentesis or Chorionic Villus Sampling (CVS): These are invasive prenatal tests that can detect certain genetic abnormalities associated with CHDs. However, they are typically only recommended if there's a high risk based on family history or other factors.

If a baby is born with cyanotic symptoms like bluish skin (cyanosis), rapid breathing, or feeding difficulties, a prompt and thorough evaluation is essential. Several tests help pinpoint the underlying CHD:

• Physical Examination: The doctor will examine the baby for signs like cyanosis, clubbing of fingers and toes, and heart murmurs (abnormal sounds heard through a stethoscope).
• Pulse Oximetry: This non-invasive test uses a clip placed on a finger or toe to measure the oxygen saturation in the blood. Low oxygen levels point towards a potential problem.
• Chest X-Ray: This X-ray image of the chest can reveal abnormalities in the heart size, shape, or lung appearance, which might suggest a CHD.
• Electrocardiogram (ECG): This test measures the electrical activity of the heart and can detect abnormal heart rhythms associated with certain CHDs.
• Echocardiogram: This remains the cornerstone of diagnosis after birth. It provides detailed images of the heart's structure, blood flow patterns, and valve function, allowing doctors to pinpoint the specific CHD and its severity.
• Cardiac Catheterization: In some complex cases, a thin tube (catheter) is inserted into a blood vessel and guided to the heart. This allows for direct measurement of pressures within the heart chambers and blood vessels, providing valuable information about the specific defect and blood flow patterns.

Atrial septostomy, whether performed via balloon catheter or surgery, involves several distinct stages. Here's a breakdown of each stage for both procedures:

Rashkind Balloon Septostomy Stages:

1. Pre-procedural Preparation:

• Medical evaluation: Your doctor will assess your overall health, including medical history, medications, and allergies.
• Informed consent: You'll be thoroughly informed about the procedure, its risks and benefits, and alternative options.
• Fasting: You might be instructed to stop eating and drinking a certain number of hours before the procedure.
• IV line insertion: An intravenous (IV) line will be inserted in your child's arm or leg for administering fluids and medications.

2. Catheterization Procedure:

• Anaesthesia: General anaesthesia is administered to keep your child asleep and comfortable throughout the procedure.
• Catheter insertion: A thin, flexible tube (catheter) is inserted into a vein in the groyne and guided through the blood vessels to the right atrium of the heart. This is usually done with the help of fluoroscopy (real-time X-ray imaging).
• Balloon positioning: The catheter tip with a small balloon is carefully manoeuvred into the foramen ovale, the natural opening between the atria that usually closes shortly after birth.
• Balloon inflation and septostomy creation: The balloon is inflated momentarily, enlarging the foramen ovale and creating a permanent opening in the atrial septum.
• Catheter removal: Once the septostomy is created, the balloon is deflated and the catheter is carefully removed.

3. Post-procedural Care:

• Monitoring: Your child will be closely monitored in a recovery room for vital signs, heart function, and any potential complications.
• Pain management: Medication will be provided to manage any discomfort your child might experience.
• Recovery and discharge: Once stable, your child will be transferred to a regular room for continued observation. Depending on their condition, they might be discharged home within a day or two.

Surgical Septostomy Stages:

1. Pre-surgical Preparation:

• Medical evaluation: Similar to the balloon procedure, a thorough medical evaluation will be conducted.
• Informed consent: You'll be given detailed information about the surgery, risks, benefits, and recovery process.
• Pre-operative tests: Additional tests like chest X-ray and blood tests might be ordered.
• Fasting: Fasting instructions will be provided based on the surgery schedule.

2. Surgical Procedure:

• General anaesthesia: General anaesthesia is administered to keep you or your child unconscious during the surgery.
• Incision: A surgical incision is made in the chest to access the heart.
• Septostomy creation: The surgeon directly creates a controlled opening in the atrial septum.
• Closure and drainage: The incision is then closed with sutures, and a chest tube might be inserted to drain any excess fluids from around the heart.

3. Post-surgical Care:

• Intensive care unit (ICU): Following surgery, you or your child will be transferred to the ICU for close monitoring of vital signs and recovery.
• Pain management: Medication will be provided to manage pain and discomfort.
• Breathing support: A ventilator might be used to assist with breathing initially.
• Gradual recovery: Over time, breathing support will be gradually reduced, and you or your child will be transitioned to a regular room.
• Physical therapy: Depending on the complexity of the surgery, physical therapy might be recommended to improve strength and mobility during recovery.
• Hospital discharge: Once stable, you or your child will be discharged home with specific instructions for recovery and follow-up care.

The road to recovery after atrial septostomy depends on several factors, including the type of procedure performed (balloon septostomy vs. surgical septostomy), the underlying heart defect, and the baby's overall health. Here's a breakdown of what to expect:

Immediately After the Procedure:

• In the Catheterization Lab (Rashkind Septostomy): Following a balloon septostomy, your baby will be monitored for a few hours in the catheterization lab. Nurses will check vitals, heart function, and the groyne insertion site for any bleeding or complications. Once stable, your baby can usually return to the nursery or paediatric intensive care unit (PICU).
• Recovery Room (Surgical Septostomy): After open-heart surgery, your baby will be transferred to the recovery room for close monitoring. Here, they'll be on a ventilator to assist breathing and have various tubes and catheters to drain fluids and monitor vitals. Pain medication will be administered to manage discomfort.

Hospital Stay:

• The length of your baby's hospital stay will vary depending on the procedure and their recovery progress. For a balloon septostomy, the stay might be as short as 24-48 hours. For surgical septostomy, it could be several days or even a week.
• During this time, nurses will monitor your baby's vitals, heart function, oxygen levels, and surgical site for any signs of infection. They will also gradually wean your baby off ventilator support and intravenous fluids as they recover.
• You'll be educated on caring for your baby's incision site, monitoring for signs of infection, and administering medications if prescribed.

At Home Recovery:

• Once discharged, continued monitoring and care are crucial for your baby's well-being. Here's what to expect:
  • Medications: Your baby might need medications to manage heart rhythm, prevent infection, or address any underlying conditions.
  • Feeding: Feeding difficulties are common after heart procedures. Your doctor will advise on appropriate feeding techniques and monitor your baby's weight gain.
  • Activity: Initially, restricted activity might be recommended to allow for healing. Gradually, your baby can resume normal activities as tolerated.
  • Follow-up Appointments: Regular follow-up appointments with your paediatrician and cardiologist are essential to monitor your baby's progress and assess the effectiveness of the atrial septostomy.

1. What is atrial septostomy?
   Atrial septostomy is a procedure used to create or enlarge an opening in the atrial septum, the wall between the two upper chambers of the heart (atria).
2. Why is atrial septostomy performed?
   It is performed to relieve symptoms of certain heart conditions, such as pulmonary hypertension or congenital heart defects, by improving blood flow and oxygenation.
3. How is atrial septostomy performed?
   The procedure is usually done using a catheter, a thin tube inserted into a blood vessel and guided to the heart. A special tool is then used to create or enlarge the opening in the atrial septum.
4. Is atrial septostomy a major surgery?
   No, atrial septostomy is typically considered a minimally invasive procedure.
5. What conditions may require atrial septostomy?
   Conditions such as severe pulmonary hypertension, transposition of the great arteries, and other congenital heart defects may necessitate atrial septostomy.
6. Who performs atrial septostomy?
   Interventional cardiologists, who specialise in minimally invasive procedures to treat heart conditions, typically perform atrial septostomy.
7. Is atrial septostomy safe?
   While all medical procedures carry some risks, atrial septostomy is generally considered safe when performed by experienced medical professionals.
8. What are the risks associated with atrial septostomy?
   Risks may include bleeding, infection, arrhythmias, stroke, or damage to surrounding structures in the heart.
9. How long does atrial septostomy take?
   The duration of the procedure varies but typically lasts between one to three hours.
10. Is atrial septostomy painful?
    Patients are usually sedated or under general anaesthesia during the procedure, so they do not experience pain.
11. What happens after atrial septostomy?
    Patients are usually monitored closely for a period after the procedure and may require medication or further interventions depending on their condition.
12. How long is the recovery period after atrial septostomy?
    Recovery times vary but patients may be discharged from the hospital within a day or two after the procedure.
13. Are there any restrictions after atrial septostomy?
    Patients may be advised to avoid strenuous activity for a period after the procedure, but specific restrictions will depend on individual circumstances.
14. Can atrial septostomy cure pulmonary hypertension?
    Atrial septostomy can improve symptoms of pulmonary hypertension but may not cure the underlying condition. It is often done as a palliative measure to alleviate symptoms.
15. What are the benefits of atrial septostomy?
    Benefits may include improved oxygenation, reduced symptoms such as shortness of breath and fatigue, and improved quality of life for patients with certain heart conditions.
16. Are there alternatives to atrial septostomy?
    Depending on the specific condition, alternatives may include medication, other surgical procedures, or heart transplantation.
17. Can atrial septostomy be performed in children?
    Yes, atrial septostomy can be performed in paediatric patients with certain congenital heart defects or pulmonary hypertension.
18. How long has atrial septostomy been performed?
    Atrial septostomy has been performed for several decades, with refinements in techniques and equipment over time.
19. Is atrial septostomy covered by insurance?
    Insurance coverage for atrial septostomy may vary depending on the patient's insurance plan and medical necessity.
20. What is the success rate of atrial septostomy?
    Success rates vary depending on the underlying condition and other factors but are generally high when performed by experienced practitioners.
21. Can atrial septostomy be performed more than once?
    In some cases, atrial septostomy may need to be repeated if the initial procedure does not provide sufficient relief or if the condition worsens over time.
22. Can atrial septostomy be performed during pregnancy?
    Atrial septostomy during pregnancy is rare and typically only considered in life-threatening situations where the benefits outweigh the risks to both the mother and the foetus.
23. How soon after birth can atrial septostomy be performed in infants?
    Atrial septostomy in infants may be performed shortly after birth if necessary, depending on the severity of the condition.
24. Are there any long-term complications of atrial septostomy?
    While uncommon, potential long-term complications may include heart rhythm abnormalities, recurrent symptoms, or the need for additional interventions.
25. Can atrial septostomy be performed in patients with other heart conditions?
    Atrial septostomy may be considered in patients with certain other heart conditions, but individual evaluation is necessary to determine suitability.
26. What is the cost of atrial septostomy?
    The cost of atrial septostomy can vary widely depending on factors such as location, hospital charges, and insurance coverage.
27. How is recovery monitored after atrial septostomy?
    Recovery is typically monitored through regular follow-up appointments with the cardiologist and may include tests such as echocardiograms or electrocardiograms.
28. Is atrial septostomy reversible?
    In some cases, the effects of atrial septostomy may be reversible through additional procedures or interventions.
29. Can atrial septostomy be performed in elderly patients?
    Atrial septostomy may be considered in elderly patients with certain heart conditions, but individual evaluation is necessary to assess risks and benefits.
30. Can complications occur during atrial septostomy?
    While rare, complications such as cardiac perforation, embolism, or arrhythmias can occur during the procedure.
31. Does atrial septostomy require a hospital stay?
    Most patients require a short hospital stay for monitoring after atrial septostomy, but the duration may vary depending on individual circumstances.
32. Can atrial septostomy be performed without anaesthesia?
    Atrial septostomy is typically performed under sedation or general anaesthesia to ensure patient comfort and safety.
33. How is the size of the atrial septal defect determined before atrial septostomy?
    The size of the defect is usually assessed using imaging techniques such as echocardiography or cardiac catheterization before the procedure.
34. Are there any dietary restrictions after atrial septostomy?
    Unless otherwise advised by a healthcare provider, patients typically do not have specific dietary restrictions after atrial septostomy.
35. How soon can normal activities be resumed after atrial septostomy?
    Patients may gradually resume normal activities as tolerated, but individual recovery times may vary.
36. Can atrial septostomy improve exercise tolerance?
    Atrial septostomy may improve exercise tolerance in patients with certain heart conditions by increasing oxygenation and blood flow.
37. What are the signs that atrial septostomy may be necessary?
    Signs that atrial septostomy may be necessary include severe shortness of breath, fatigue, cyanosis (bluish discoloration), and other symptoms of inadequate oxygenation.
38. Can atrial septostomy be performed on an outpatient basis?
    Atrial septostomy is typically performed in a hospital setting, and most patients require at least an overnight stay for monitoring.
39. Can atrial septostomy be performed in patients with heart failure?
    Atrial septostomy may be considered in carefully selected patients with heart failure, but individual evaluation is necessary to assess risks and benefits.
40. How is the success of atrial septostomy measured?
    The success of atrial septostomy is typically measured by improvements in symptoms, oxygenation levels, and overall quality of life for the patient.
41. Can atrial septostomy be performed in patients with blood clotting disorders?
    Atrial septostomy may be performed in patients with blood clotting disorders, but precautions will be taken to minimise the risk of bleeding complications.
42. What is the role of imaging in planning atrial septostomy?
    Imaging techniques such as echocardiography and cardiac MRI are used to assess the anatomy of the heart and guide the planning of atrial septostomy.
43. Are there any age limitations for atrial septostomy?
    Atrial septostomy may be considered in patients of all ages, but individual evaluation is necessary to assess suitability and risks.
44. Can atrial septostomy be performed in patients with pacemakers?
    Atrial septostomy may be performed in patients with pacemakers, but careful coordination with the cardiology team is necessary to ensure safety and efficacy.
45. How long do the effects of atrial septostomy last?
    The effects of atrial septostomy may vary depending on the underlying condition but can be long-lasting in some cases.
46. Can atrial septostomy be performed in patients with other medical conditions?
    Atrial septostomy may be considered in patients with other medical conditions, but individual evaluation is necessary to assess risks and benefits.
47. Can atrial septostomy be performed in patients with a history of heart surgery?
    Atrial septostomy may be considered in patients with a history of heart surgery, but individual evaluation is necessary to assess risks and benefits.
48. Can atrial septostomy be performed in patients with valve disorders?
    Atrial septostomy may be considered in patients with valve disorders, but individual evaluation is necessary to assess risks and benefits.
49. How is atrial septostomy different from other heart procedures?
    Atrial septostomy specifically targets the atrial septum to improve blood flow and oxygenation, whereas other procedures may target different aspects of heart function or anatomy.
50. Is atrial septostomy suitable for all patients with heart conditions?
    Atrial septostomy may be suitable for certain patients with specific heart conditions, but individual evaluation by a cardiologist is necessary to determine suitability and risks.