Differential Diagnosis for Increased RAP after Glenn Procedure
Single Most Likely Diagnosis
- Pulmonary Arteriovenous Malformations (PAVMs): The Glenn procedure, which involves diverting blood flow from the superior vena cava directly to the pulmonary arteries, can sometimes lead to the development of PAVMs. These malformations can cause increased right atrial pressure (RAP) due to abnormal blood flow and resistance in the pulmonary vasculature.
Other Likely Diagnoses
- Pulmonary Hypertension: Elevated pulmonary vascular resistance can increase the pressure in the right atrium, especially after a Glenn procedure, where the right heart is partially bypassed but still subject to changes in pulmonary vascular resistance.
- Anastomotic Stenosis or Obstruction: Narrowing or blockage at the site where the superior vena cava is anastomosed to the pulmonary artery can increase resistance to blood flow, leading to elevated RAP.
- Right Ventricular Dysfunction: Although the Glenn procedure reduces the workload on the right ventricle, any residual or developing dysfunction can still lead to increased RAP due to the ventricle's inability to efficiently pump blood.
Do Not Miss Diagnoses
- Thrombosis: Clots in the pulmonary arteries or at the anastomotic site can severely obstruct blood flow, leading to a sudden increase in RAP. This condition is life-threatening and requires immediate intervention.
- Pulmonary Embolism: Similar to thrombosis, a pulmonary embolism can significantly increase pulmonary vascular resistance and RAP, posing a critical risk to the patient.
Rare Diagnoses
- Protein-Losing Enteropathy: Although more commonly associated with Fontan circulation, protein-losing enteropathy can also occur after a Glenn procedure. It leads to hypoalbuminemia, which can cause increased capillary permeability and fluid retention, potentially increasing RAP.
- Lymphatic Dysfunction: Abnormalities in lymphatic drainage, which can be a consequence of the surgical alteration of venous return, might contribute to increased fluid accumulation and pressure in the right atrium.
- Cardiac Lymphangioma: A rare condition where lymphatic malformations involve the heart, potentially affecting the right atrium and causing increased pressure due to obstruction or fluid accumulation.