Total Anomalous Pulmonary Venous Return (TAPVR)

Total Anomalous Pulmonary Venous Return (TAPVR)

> a birth defect of the heart in which the veins bringing blood back from the lungs (pulmonary veins) don’t connect to the left atrium like usual. Instead they go to the heart by way of an abnormal (anomalous) connection.

> In a baby with TAPVR, oxygen-rich blood does not return from the lungs to the left atrium. Instead, the oxygen-rich blood returns to the right side of the heart. Here, oxygen-rich blood mixes with oxygen-poor blood.

> This causes the baby to get less oxygen than is needed to the body.

To survive with this defect, babies with TAPVR usually have a hole between the right atrium and the left atrium (an atrial septal defect) that allows the mixed blood to get to the left side of the heart and pumped out to the rest of the body. Some children can have other heart defects along with TAPVR, aside from the atrial septal defect. Because a baby with this defect may need surgery or other procedures soon after birth, TAPVR is considered a critical congenital heart defect.

Types

1. Supracardiac - In supracardiac TAPVR, the pulmonary veins come together and form an abnormal connection above the heart to the superior vena cava, which is a main blood vessel that brings oxygen-poor blood from the upper part of the body to the heart. In this type of TAPVR, a mixture of oxygen-poor and oxygen-rich blood returns to the right atrium through the superior vena cava.

2. Cardiac - In cardiac TAPVR, the pulmonary veins meet behind the heart and connect to the right atrium. The coronary sinus, which is a vein that helps bring oxygen-poor blood from the heart muscle back to the heart, helps connect the pulmonary veins to the right atrium in this type of TAPVR.

3. Infracardiac - In infracardiac TAPVR, the pulmonary veins come together and form abnormal connections below the heart. A mixture of oxygen-poor blood and oxygen-rich blood returns to the right atrium from the veins of the liver and the inferior vena cava, which is the main blood vessel that brings oxygen-poor blood from the lower part of the body to the heart.

Cause and Risk factor

The causes of heart defects, such as TAPVR, among most babies are unknown. Some babies have heart defects because of changes in their genes or chromosomes. Heart defects also are thought to be caused by a combination of genes and other risk factors, such as the things the mother or fetus come in contact with in the environment or what the mother eats or drinks or the medicines she uses.

Signs and Symptoms

1. Problems breathing

2. Pounding heart

3. Weak pulse

4. Ashen or bluish skin color

5. Poor feeding

6. Extreme sleepiness

TAPVR procedure

The goal of the surgical repair of TAPVR is to restore normal blood flow through the heart. To repair this defect, doctors usually connect the pulmonary veins to the left atrium, close off any abnormal connections between blood vessels, and close the atrial septal defect.

Nursing Responsibiliy

1. Ventilator - a machine that helps your child breathe while he/she is under anesthesia during the operation. After a TAPVR repair, children will benefit from remaining on the ventilator overnight or even longer so they can rest.

2. Intravenous (IV) catheters - to provide IV fluids and important medicines that help your child recover from the operation.

3. Arterial line - measures blood pressure continuously during surgery and while your child is in the PACU.

4. Nasogastric (NG) tube - keeps the stomach drained of acid and gas bubbles that may build up during surgery.

5. Urinary catheter - allows urine to drain out of the bladder and accurately measures how much urine the body makes, which helps determine how well the heart is functioning. Diuretics may be given to help the kidneys to remove excess fluid from the body.

6. Chest tube - a drainage tube may be inserted to keep the chest free of blood that would otherwise accumulate after the incision is closed. Bleeding may occur for several hours, or even a few days after surgery.

7. Heart monitor - a machine that constantly displays a picture of your child's heart rhythm, and monitors heart rate, arterial blood pressure, and other values.

Reference

https://www.cdc.gov/ncbddd/heartdefects/tapvr.html

https://childrenswi.org/medical-care/herma-heart/conditions/total-anomalous-pulmonary-venous-return

Supplies and Instruments

1 Cotton 2.0 (strands)

1 Feeding tube Fr.10

1 Povidone Iodine 7.5%

2 Gloves 6

2 Gloves 7

2 Gloves 7 1/2

2 Pacing wire

2 Povidone Iodine 10%

2 Silk 2.0 (strands)

3 Feeding tube Fr.8

3 Lubricating Jelly

4 Gloves 6 1/2

10 Operating Gauze

20cc syringe

Alcohol

Asepto

Blades 11 and 15

Bone wax

Cautery cord

Cautery pad (neo/pedia)

Foley catheter (age consideration)

Ioban

IV cannula G20

Marker (standby)

Monocryl 3.0

Peanut

Small horizon clips

Steel wire no.1

Sterile water 50ml

Suction tubing

Tegaderm (Large)

Thoracic drape

Urine bag

Vicryl 4.0 PC5

Drainage: JP Drain

1pc Derra

1pc Fine mixter

2pcs castañeda clamp

Basura box

Gregory (standby)

Heggars dilators 8pcs (standby)

horizon clip applier

Horizon small clip applier

Marylou

Mayo/Metz

Pedia finochietto

Pedia Open Heart prep set

Pedia Open Heart set

Pedia paddle

Pedia set no. 1

ruler (standby)

Stryker saw

Weighted sucker (drop in sucker)

Y connector 1/4 all

Doctor Reference:

1. Dr. Nicolas / Dr. Catalan

Antergrade cannula: 

- IV Gray cannula Fr. 16 (adult) 

- IV cannula Fr.18 (neo/pedia) 

Dr. Catalan's set (2sets)

Dr. Catalan's Noir metz scissor

Saw: Bottom to Top

2. Dr. Garcia

Antegrade cannula: DLP Aortic Root Medtronic

1 Silk 3.0 HR26

3pcs instruments

Saw: Bottom to Top