Anomalous pulmonary venous connection

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Anomalous pulmonary venous connection
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Illustration of total anomalous pulmonary venous connection
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Anomalous pulmonary venous connection (or anomalous pulmonary venous drainage or anomalous pulmonary venous return [1] ) is a congenital heart defect of the pulmonary veins. It can be a total anomalous pulmonary venous connection, wherein all four pulmonary veins are incorrectly positioned, or a partial anomalous pulmonary venous connection, wherein only some of the pulmonary veins are incorrectly positioned.

Contents

Signs and symptoms

Total anomalous pulmonary venous connection (TAPVR)

Total anomalous pulmonary venous connection, also known as total anomalous pulmonary venous return, is a rare cyanotic congenital heart defect in which the pulmonary veins drain into the right side of the heart instead of the left, as is usually seen. This can happen within the heart (intracardiac) where it drains into the coronary sinus or right atrium, or below it (infracardiac) where it drains into the liver at the portal or hepatic vein. The anomalous connection causes low blood oxygenation and limitation of venous return to the heart. [3]

Diagnosis

The diagnosis of TAPVC is dependent upon whether or not there is an obstruction. [4]

TAPVC with obstruction will clinically present with cyanosis, tachypnea, dyspnea, and hypoxemia. Chest x-ray may show a ground-glass appearance due to pulmonary edema resulting from increased pressure in the obstructed pulmonary veins.

Treatment

Cases of TAPVC with obstruction are considered a surgical emergency.

In TAPVC without obstruction, surgical redirection can be performed within the first month of life. The operation is performed under general anesthesia. The four pulmonary veins are reconnected to the left atrium, and any associated heart defects such as atrial septal defect, ventricular septal defect, patent foramen ovale, and/or patent ductus arteriosus are surgically closed. With obstruction, surgery should be undertaken urgently. PGE1 should be given because a patent ductus arteriosus allows oxygenated blood to go from the circulation of the right heart to the systemic circulation.[ citation needed ]

Partial anomalous pulmonary venous connection (PAPVR)

A Partial anomalous pulmonary venous connection (or Partial anomalous pulmonary venous drainage or Partial anomalous pulmonary venous return) is a congenital defect where the left atrium is the point of return for the blood from some (but not all) of the pulmonary veins.[ citation needed ]

It is less severe than total anomalous pulmonary venous connection which is a life-threatening anomaly requiring emergent surgical correction, usually diagnosed in the first few days of life. Partial anomalous venous connection may be diagnosed at any time from birth to old age. The severity of symptoms, and thus the likelihood of diagnosis, varies significantly depending on the amount of blood flow through the anomalous connections. In less severe cases, with smaller amounts of blood flow, diagnosis may be delayed until adulthood, when it can be confused with other causes of pulmonary hypertension. [5] There is also evidence that a significant number of mild cases are never diagnosed, or diagnosed incidentally. [6] It is associated with other vascular anomalies, and some genetic syndromes such as Turner syndrome.[ citation needed ]

Diagnosis

It can be diagnosed with CT scan, angiography, transesophageal echocardiography, or cardiac MRI. [7] [8]

Treatment

It is sometimes treated with surgery, which involves rerouting blood from the right atrium into the left atrium with a patch or use of the Warden procedure. [9] [10] However, interest is increasing in catheter-based interventional approaches, as well as medical therapy for less severe cases[ citation needed ].

Pathophysiology

To understand the different types of anomalous pulmonary venous connection, it is important to establish the background in which normal fetal vasculature develops.

During embryonic development, the lungs start as outgrowths from the foregut and share its splanchnic vascular plexus. At around 25 to 27 days of gestation, there are no direct links to the heart. By 27 to 29 days of gestation, the primitive pulmonary vein begins to form as an outgrowth from the left atrium and eventually connects with the primitive pulmonary venous system.

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<span class="mw-page-title-main">Congenital heart defect</span> Defect in the structure of the heart that is present at birth

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<span class="mw-page-title-main">Pulmonary vein stenosis</span> Medical condition

Pulmonary vein stenosis is a rare cardiovascular disorder. It is recognized as being the stenosis of one or more of the four pulmonary veins that return blood from the lungs to the left atrium of the heart. In congenital cases, it is associated with poor prognosis and high mortality rate. In some people, pulmonary vein stenosis occurs after pulmonary vein ablation for the treatment of atrial fibrillation. Some recent research has indicated that it may be genetically linked in congenital cases.

Isolated levocardia is a rare type of organs' situs inversus in which the heart is still in normal position but other abdominal viscera are transposed. Isolated levocardia may occur with heart defects and patients without having operations have low life expectancy: only about 5% to 13% of patients survive more than 5 years. Therefore, even though the risk of cardiac surgeries is high, once patients are diagnosed, operations are suggested to be held as soon as possible. Isolated levocardia is congenital. So far, there is not sufficient evidence to prove that chromosome abnormalities will result in isolated levocardia, and the cause of isolated levocardia is still unknown.

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. This can be considered a dangerous heart condition because it puts the individual at a high risk of stroke. Other defects that are often associated with Raghib syndrome can include ventricular septal defects, enlargement of the tricuspid annulus, and pulmonary stenosis. 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. 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.

References

  1. ↑ "Facts about Total Anomalous Pulmonary Venous Return or TAPVR" . Retrieved November 10, 2013.
  2. ↑ Somerville J, Grech V (2009). "The chest x-ray in congenital heart disease 1. Total anomalous pulmonary venous drainage and coarctation of the aorta". Images Paediatr Cardiol. 11 (1): 7–9. ISSN   1729-441X. PMC   3232600 . PMID   22368552.
  3. ↑ Byard, Roger W.; Gilbert, John D. (2005-09-01). "Total anomalous pulmonary venous connection". Forensic Science, Medicine, and Pathology. 1 (3): 215–220. doi:10.1385/FSMP:1:3:215. ISSN   1556-2891.
  4. ↑ Stein, Patricia (March 2007). "Total Anomalous Pulmonary Venous Connection". AORN Journal. 85 (3): 509–520. doi:10.1016/S0001-2092(07)60123-9. ISSN   0001-2092.
  5. ↑ Sears EH, Aliotta JM, Klinger JR (2012). "Partial anomalous pulmonary venous return presenting with adult-onset pulmonary hypertension". Pulm Circ. 2 (2): 250–5. doi: 10.4103/2045-8932.97637 . PMC   3401879 . PMID   22837866.
  6. ↑ Ho ML, Bhalla S, Bierhals A, Gutierrez F (May 2009). "MDCT of partial anomalous pulmonary venous return (PAPVR) in adults". J Thorac Imaging. 24 (2): 89–95. doi:10.1097/RTI.0b013e318194c942. PMID   19465830.
  7. ↑ Prasad SK, Soukias N, Hornung T, Khan M, Pennell DJ, Gatzoulis MA, et al. (2004). "Role of magnetic resonance angiography in the diagnosis of major aortopulmonary collateral arteries and partial anomalous pulmonary venous drainage". Circulation. 109 (2): 207–14. doi: 10.1161/01.cir.0000107842.29467.c5 . PMID   14718402.
  8. ↑ Ammash NM, Seward JB, Warnes CA, Connolly HM, O'Leary PW, Danielson GK (May 1997). "Partial anomalous pulmonary venous connection: diagnosis by transesophageal echocardiography". J. Am. Coll. Cardiol. 29 (6): 1351–8. doi: 10.1016/S0735-1097(97)82758-1 . PMID   9137235.
  9. ↑ ElBardissi AW, Dearani JA, Suri RM, Danielson GK (March 2008). "Left-sided partial anomalous pulmonary venous connections". Ann. Thorac. Surg. 85 (3): 1007–14. doi:10.1016/j.athoracsur.2007.11.038. PMID   18291189.
  10. ↑ Alsoufi B, Cai S, Van Arsdell GS, Williams WG, Caldarone CA, Coles JG (December 2007). "Outcomes after surgical treatment of children with partial anomalous pulmonary venous connection". Ann. Thorac. Surg. 84 (6): 2020–6, discussion 2020–6. doi: 10.1016/j.athoracsur.2007.05.046 . PMID   18036929.
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