Raghib syndrome

Raghib syndrome is rare a congenital heart defect where the left superior vena cava (LSVC) is draining into the left atrium in addition to an absent coronary sinus and an atrial septal defect.[1][2] This can be considered a dangerous heart condition because it puts the individual at a high risk of stroke.[3] Other defects that are often associated with Raghib syndrome can include ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis.[3] While this is considered an extremely rare developmental complex, cases regarding a persistent left superior vena cava (PLSVC) are relatively common among congenital heart defects.[4][3] It is also important to note that the PLSVC often drains into the right atrium, and only drains into the left atrium in approximately 10 to 20% of individuals with the defect.[5]

In an individual with no underlying heart condition, the superior vena cava delivers blood returning from the body to the right atrium.[6] Due to oxygen being used by various biological processes throughout the body, this blood has extremely low blood oxygenation levels.[6] Therefore, the right side of the heart pumps this blood to the lungs where gas exchange takes place in the capillaries.[7] The oxygenated blood is then transported back to the left side of the heart through pulmonary veins.[7] The left atrium and left ventricle work to push the now oxygen saturated blood up through the aorta and out to the body.[7] As stated earlier, individuals with Raghib syndrome experience drainage from the left superior vena cava into the left atrium.[1] This means that the deoxygenated blood returning from the body is directly bypassing the lungs and entering the left atrium where it will be pumped back into the body causing cyanosis.[5] The result of this can have major implications on several biological processes, often requiring surgical intervention.[5]

The coronary sinus is a vein continuing off of the great cardiac vein.[8] It collects blood from the ventricular veins of the heart muscle during ventricle contraction and moves this blood into the right atrium.[8] Essentially, this coronary sinus takes de-oxygenated blood from veins in the heart muscle (epicardial ventricular veins) and delivers it to the right side of the heart so it can be transported to the lungs and oxygenated again.[8] It is extremely rare for an individual to be lacking this component of the heart.[9]

Lastly, a common aspect of Raghib syndrome is an atrial septal defect. This is when there is a hole in the septum dividing the right and left atriums of the heart.[10] The size of this hole may vary significantly from individual to individual [10] Some may not even notice the symptoms of this defect until they are well into adulthood, if at all.[10] The main concern with an atrial septal defect, is it can cause the right side of the heart to become overworked.[11] This is because the amount of blood being pumped to the lungs increases significantly as blood from the left atrium leaks back into the right atrium.[11] Thus leading to damaged blood vessels, increased blood pressure, and can even increase the right side of the heart.[11]

  1. ^ a b Yalçınkaya, Adnan; Diken, Adem; Özkan, Muhammed; Lafçı, Gökhan; Çiçek, Ömer; Çağlı, Kerim (2013). "Raghib's syndrome with the absence of the right superior vena cava" (PDF). Turkish Journal of Thoracic and Cardiovascular Surgery. 21 (4): 1148–1149. doi:10.5606/tgkdc.dergisi.2013.8398. Retrieved 19 November 2022.
  2. ^ Pérez-Caballero, Ramón; Plata Izquierdo, Beatriz; Gil-Jaurena, Juan-Miguel (Jan 2016). "Raghib Syndrome. Surgical Treatment". Sociedad Española de Cardiología. 69 (1): 71. doi:10.1016/j.rec.2015.04.002. PMID 26190545. S2CID 34059619. Retrieved 11 November 2022.
  3. ^ a b c Daruwalla, Vistasp; Parekh, Keyur; Tahir, Hassan; Collins, Jeremy; Carr, James (27 May 2015). "Raghib Syndrome Presenting as a Cryptogenic Stroke: Role of Cardiac MRI in Accurate Diagnosis". Case Rep Cardiol. 2015: 921247. doi:10.1155/2015/921247. PMC 4461698. PMID 26106490.
  4. ^ Azizova, Aynur; Onder, Omer; Arslan, Sevtap; Ardali, Selin; Hazirolan, Tuncay (15 Oct 2020). "Persistent left superior vena cava: clinical importance and differential diagnoses". Insights into Imaging. 11 (1): 110. doi:10.1186/s13244-020-00906-2. PMC 7561662. PMID 33057803.
  5. ^ a b c Tyrak, Kamil; Hołda, Mateusz; Koziej, Mateusz; Piątek, Katarzyna; Klimek-Piotrowska, Wiesława (2017). "Persistent left superior vena cava". Cardiovasc J Afr. 28 (3): e1–e4. doi:10.5830/CVJA-2016-084. PMC 5558145. PMID 28759082.
  6. ^ a b White, Hunter; Soos, Michael (Jan 2022). "Anatomy, Thorax, Superior Vena Cava". StatPearls Publishing. PMID 31424839. Retrieved 19 November 2022. {{cite journal}}: Cite journal requires |journal= (help)
  7. ^ a b c "How Blood Flows through the Heart". National Heart, Lung, and Blood Institute. Retrieved 28 November 2022.
  8. ^ a b c Habib, Ammar; Lachman, Nirusha; Christensen, Kevin; Asirvatham, Samuel (1 Nov 2009). "The anatomy of the coronary sinus venous system for the cardiac electrophysiologist". EP Europace. 11 (5): v15–v21. doi:10.1093/europace/eup270. PMID 19861386.
  9. ^ Yolcu, Mustafa; Turkmen, Serdar; Sertcelik, Alper; Yolcu, Canan; Batyraliev, Talant (25 Nov 2013). "Isolated Absence of Coronary Sinus: Two Cases Report". J Clin Diagn Res. 7 (12): 3006–3007. doi:10.7860/JCDR/2013/6431.3829. PMC 3919330. PMID 24551710.
  10. ^ a b c "Facts about Atrial Septal Defect". Centers for Disease Control and Prevention. 19 November 2019. Retrieved 28 November 2022.
  11. ^ a b c "Atrial septal defect (ASD)". Mayo Clinic. Retrieved 28 November 2022.