PAPVR Partial Anomalous Pulmonary Venous Return

Partial Anomalous Pulmonary Venous Return

(abbr. PAPVR) anomalous pulmonary venous connection, when at least at least one pulmonary vein connected to the right atrium either directly or indirectly through a venous tributary.

TAPVR Total Anomalous Pulmonary Venous Return

Total Anomalous Pulmonary Venous Return

(abbr. TAPVR) - rare congenital cardiac anomaly in a 1,5% of children born with congenital heart defects. In simple words it's anomalous pulmonary venous connection.

Types of Total Anomalous Pulmonary Venous Return

1. Supracardiac
2. Cardiac
3. Infracardiac
4. Mixed

Video

by Redmond Burke MD from Miami Children's Hospital
3D CT images of TAPVR

TOF - Tetralogy of Fallot

TOF - Tetralogy of Fallot

- congenital heart disease, including four defects.

History of TOF

Video

by Dr. Redmond Burke from Miami Children's Hospital

Truncus Arteriosus

Truncus Arteriosus

also Common Arterial Trunk

Video

Video of operation for Truncus Arteriosus by Dr. Redmond Burke

External resourses:

* Truncus Arteriosus - eMedicine
* Truncus Arteriosus - Cincinati Children's

Coarctation of the Aorta

Coarctation of the Aorta

also Aortic Coarctation is a narrowing of the aorta

History

Video

by Dr. Redmond Burke from Miami Children's Hospital

IAA Interrupted Aortic Arch

Interrupted Aortic Arch

(abbr. IAA)usually occurs in association with a large ventricular septal defect and patent ductus arteriosus or with a large aortopulmonary window or truncus arteriosus.

HLHS Hypoplastic Left Heart Syndrome

Hypoplastic Left Heart Syndrome

- HLHS - clinical syndrome, characterized by congenital heart disease such as mild or severe hypoplasia or atresia of the mitral valve, left ventricle, aortic valve hypoplasia and ascending aorta. Cardiac output goes from the right ventricle through the patent ductus arteriosus (PDA) to the aorta. Death of patients due to the closure of PDA occurs in the first days or weeks of life.

History

• 1952 - Aortic hypoplasia in combination with birth defects such as Ventricular septal defect, Aortic stenosis or atresia, Mitral stenosis or atresia was first described by M. Lev.
• 1958 - Hypoplastic Left Heart Syndrome first described in details by J. Noonan and A. Nadas, but only as a pathological diagnosis.
• 1983 - American surgeon Bill Norwood from Children's Hospital Boston published the results of surgical operations in neonates with HLHS, who provided a chance for a healthy life to these patients. In the future this type of operation was named after him.
• 1991 - doctors in UK London Guy's Hospital, led by D. Maxwell made the world's first fetal balloon valvuloplasty of aortic valve in two fetuses in the womb with marked aortic stenosis under echocardiography control. By doing so they opened the prospect of treatment HLHS in unborn children.

Surgical treatment

Surgical treatment of HLHS includes three stages:
The first phase - Norwood operation.
Performed usually under 14 days. It provided:
• The smooth flow of the systemic ventricle.
• Adequate and unobstructed coronary circulation.
• The systemic-pulmonary arterial shunt in parallel to ensure smooth and pulmonary circulation interatrial connection for smooth pulmonary venous return.
Possible implementation of the Modified Norwood operation, first proposed by Japanese cardiac surgeon Shunji Sano.
Some patients after the first stage of surgical treatment may require temporary support of heart function by extracorporeal membrane oxygenation or ventricular assist device.
The second phase - Bidirectional cavopulmonary anastomosis or Hemi-Fontan procedure.
Performed in age from 2.5 to 4 months
The third (final) stage - Total cavopulmonary connection.
Performed at the age of 2 to 3 years (weight 12-15 kg patient).

Video by Dr. Redmond Burke for HLHS surgery: palliative first stage approach


Echocardiogram HLHS

More about Hypoplastic Left Heart Syndrome

- Surgical management of hypoplastic left heart syndrome at the Birmingham Children's Hospital by A.M. Fabricius, T.J. Jones, J. Stickley, O. Stümper, A. Chikermane, T. Desai, P. Miller, R. Dhillon, J.V. de Giovanni, J.G. Wright, D.J. Barron, W.J. Brawn MMCTS. doi:10.1510/mmcts.2006.002378.
- Surgical management of hypoplastic left heart syndrome at the Birmingham Children's Hospital by William Brawn (mp3 podcast)

HLHS review by Dr Golovenko

Single Ventricle

Single Ventricle

ccTGA - Congenitally Corrected Transposition Great Arteries

ccTGA - Congenitally Corrected Transposition Great Arteries

TGA - Transposition of the Great Arteries

TGA - Transposition of the Great Arteries - is a cyanotic congenital heart defect in which the the aorta and pulmonary artery are transposed.

VSD - Ventricular Septal Defect

Ventricular Septal Defect (VSD) is a hole between right and left ventricles. It's a tissue defect in interventricular septum.

Video

AVSD - Atrioventricular Septal Defect

AVSD - Atrioventricular Septal Defect

PDA - Patent Ductus Arteriosus

PDA - Patent Ductus Arteriosus

PDA - History

1938 - American surgeon Gross successfully ligated PDA in 7y girl at Boston Children's Hospital.

PDA - External resourses

Patent Ductus Arteriosus eMedicine,
Patent Ductus Arteriosus Mayo Clinic

ASD - Atrial Septal Defect

Atrial Septal Defect (ASD)

Pulmonary Hypertension

Pulmonary Hypertension - disorder with increase of arterial pressure in Pulmonary Artery.

Pulmonary Artery Banding

Pulmonary Artery Banding

(PA Banding) - palliative procedure in pediatric cardiac surgery performed by congenital heart surgeons in patients with congenital heart diseases and high pulmonary arterial hypertension. Today PA Banding is also a staged approach to operative correction of congenital heart defects: for example, bilateral pulmonary artery banding with PDA stenting in Hypoplastic Left Heart Syndrome.

History

1951 - Muller and Dammann in the United States performed Pulmonary Artery Banding as palliative procedure in child with a big Ventricular Septal Defect [1]

Something More about Pulmonary Artery Banding

Presentation

Video

Pulmonary Artery Banding for palliation of Hypoplastic Left Heart Syndrome

References

1. Muller WH, Dammann JF. Treatment of certain congenital malformations of the heart by the creation of pulmonic stenosis to reduce pulmonary hypertension and excessive pulmonary blood flow: A preliminary report. Surgery Gynecol Obstet. 1952;95:213 [No Abstract, No Free Full Text]