نظرة عامة

الانسداد الرئوي الخلقي المصحوب بعيب الحاجز البطيني (VSD) أحد مشكلات القلب التي توجد منذ الولادة. أي أنها عيب خلقي في القلب.

في هذا النوع من الانسداد الرئوي الخلقي، يكون الصمام الموجود بين القلب والرئتين غير مكتمل النمو. ويُطلق على هذا الصمام اسم الصمام الرئوي. ونتيجة لذلك لا يمكن للدم التدفق من حجرة القلب السفلية اليمنى، التي تُسمى البطين الأيمن، إلى الرئتين. في الانسداد الرئوي الخلقي المصحوب بعيب الحاجز البطيني، يوجد أيضًا ثقب بين حجرتي القلب المسؤولتين عن ضخ الدم.

يسمح عيب الحاجز البطيني للدم بالتدفق إلى داخل حجرة القلب السفلية اليمنى وخارجها. وقد يتدفق بعض الدم أيضًا عبر فتحة طبيعية تُسمى القناة الشريانية. وعادةً ما تنغلق القناة الشريانية أيضًا بعد الولادة بفترة قصيرة. لكن يمكن للأدوية أن تبقيها مفتوحة.

قد يكون حجم الشريان الرئوي وفروعه ضئيلاً للغاية أو لا وجود له لدى الأطفال الرُّضع المصابين بالانسداد الرئوي الخلقي. وفي حال عدم وجود هذه الأوعية الدموية، تنشأ شرايين أخرى على الشريان الرئيسي للجسم، الذي يُسمى بالشريان الأورطي. تساعد الشرايين على مواصلة تدفق الدم إلى الرئتين. وتُسمى الشرايين الأورطية الرئوية الجانبية الرئيسية (MAPCA).

الانسداد الرئوي الخلقي المصحوب بعيب الحاجز البطيني من الحالات المهددة للحياة. حيث لا يحصل الطفل المصاب بالانسداد الرئوي الخلقي على القدر الكافي من الأكسجين. وتوجد حاجة إلى تناول أدوية أو الخضوع لإجراء علاجي أو جراحة واحدة أو أكثر لتحسين تدفق الدم وإصلاح القلب.

الأنواع

  1. رتق الرئة مع سلامة الحاجز البطيني
  2. رتق الرئة مع وجود عيب في الحاجز البُطيني

الأعراض

Symptoms of pulmonary atresia with ventricular septal defect, also called PA-VSD, may appear at birth or very soon after. They can include:

  • Blue or gray skin. This change may be harder or easier to see depending on skin color.
  • Fast breathing or shortness of breath.
  • Tiredness.
  • Poor feeding.

When to see a doctor

Pulmonary atresia with ventricular septal defect, also called PA-VSD, is typically found during pregnancy or soon after birth. If your baby has symptoms of this condition after birth, call a healthcare professional right away.

الأسباب

The cause of pulmonary atresia with ventricular septal defect, also called PA-VSD, is not clear. Most congenital heart conditions happen during the first six weeks of pregnancy. The major blood vessels that run to and from the heart also begin to grow at this time. This is when a congenital heart defect such as pulmonary atresia may occur.

In PA-VSD, the pulmonary valve isn't fully formed. There also is a hole in the heart called a ventricular septal defect. The hole lets blood flow into and out of the right lower heart chamber. Some blood also may flow through a natural opening called the ductus arteriosus. The ductus arteriosus usually closes soon after birth. But medicines can keep it open.

In babies with pulmonary atresia, the lung arteries can be very small. Or they may be missing. If the blood vessels are missing, other vessels form on the body's main artery, called the aorta. These new vessels help send blood to the lungs. They are called major aortopulmonary collateral arteries, also called MAPCAs.

عوامل الخطورة

It's not clear what increases the risk of pulmonary atresia with ventricular septal defect. Possible risk factors for congenital heart conditions in general may include:

  • Smoking. If you smoke, quit. Smoking during pregnancy or being around cigarette smoke increases the risk of some congenital heart conditions.
  • Alcohol use. Drinking alcohol during pregnancy may increase the risk of heart conditions in the baby.
  • Some medicines. Some medicines taken during pregnancy may increase the risk of congenital heart conditions. These include lithium (Lithobid) for bipolar disorder and isotretinoin (Claravis, Myorisan, others), which is used to treat acne. Talk with your healthcare team about the medicines you take.
  • Genetics. Changes in some genes may affect how a baby's heart forms. For example, people with Down syndrome are often born with heart conditions.
  • Diabetes. Having type 1 or type 2 diabetes during pregnancy may change how a baby's heart forms. Diabetes that develops during pregnancy is called gestational diabetes. It typically doesn't increase a baby's risk of congenital heart conditions.
  • Rubella, also called German measles. Having rubella during pregnancy can change how a baby's heart forms. A blood test can be done before pregnancy to see if you're immune to rubella. If you're not, a vaccine is available.

24/07/2024

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