Cyanosis surgery

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief:

Cardiac catheterization

• Cardiac catheter interventions can be palliative by improving cyanosis or be corrective by relieving obstruction to flow.
• Balloon valvuloplasty can be effective in patients with critical pulmonary stenosis or aortic stenosis. Selected patients with pulmonary atresia are also candidates for balloon valvuloplasty if the obstruction is membranous, the tricuspid annulus and right ventricular size are adequate to support a two ventricle repair, and the coronary circulation does not depend upon the right ventricle [10].

Pulmonary atresia

• Cardiac catheterization is done to evaluate the defect or defects of the heart; this procedure is much more invasive.
• The patient will need to have a series of surgeries to improve the blood flow permanently.
• The type of surgery recommended depends on the size of the right ventricle and the pulmonary artery. If they are normal in size and the right ventricle is able to pump blood, open heart surgery can be performed to make blood flow through the heart in a normal pattern. If the right ventricle is small and unable to act as a pump, doctors may perform another type of operation called the Fontan procedure. In this two-stage procedure, the right atrium is disconnected from the pulmonary circulation. The systemic venous return goes directly to the lungs, by-passing the heart. The first surgery will likely be performed shortly after birth. A shunt can be created between the aorta and the pulmonary artery to help increase blood flow to the lungs. As the child grows, so does the heart and the shunt may need revised in order to meet the body's requirements.
• A cardiac catheterization procedure can be used as a diagnostic procedure, as well as initial treatment procedure as balloon atrial septostomy to improve mixing oxygenated blood and unoxygenated blood between the right and left atria.
• Atrial Septostomy: a special catheter with a balloon in the tip is used to create an opening in the atrial septum. The catheter is guided through the foramen ovale to the left atrium (LA). Once the ballon is in the LA is inflated and then pulled back opening a bigger hole between the right atrium and the LA to mix blood.
• If the hospital does not have a catheterization lab with skill physician to perform the ballon atrial septostomy, an intravenous medication called prostaglandin is administered to keep the ductus arteriousus from closing.

Transposition of great arteries

Palliative interventions

• Cardiac catheterization
  • Rashkind balloon atrial septostomy
  • Balloon angioplasty
  • Endovascular stenting
  • Angiography

Cardiac catheterization is a minimally invasive procedure which provides a means of performing a number of other procedures.

• A balloon atrial septostomy is performed with a balloon catheter, which is inserted into a foramen ovale, PFO, or ASD and inflated to enlarge the opening in the atrial septum; this creates a shunt which allows a larger amount of red blood to enter the systemic circulation.
• Angioplasty also requires a balloon catheter, which is used to stretch open a stenotic vessel; this relieves restricted blood flow, which could otherwise lead to CHF.
• An endovascular stent is sometimes placed in a stenotic vessel immediately following a balloon angioplasty to maintain the widened passage.
• Angiography involves using the catheter to release a contrast medium into the chambers and/or vessels of the heart; this process facilitates examining the flow of blood through the chambers during an echocardiogram, or shows the vessels clearly on a chest x-ray, MRI, or CT scan - this is of particular importance, as the coronary arteries must be carefully examined and "mapped out" prior to the corrective surgery.

It is commonplace for any of these palliations to be performed on a TGA patient.

Moderate

• Left anterior thoracotomy
  • Isolated pulmonary artery banding (PAB)
• Left lateral thoracotomy
  • PAB (when coarctation or aortic arch repair also required)
• Right lateral thoracotomy
  • Blalock-Hanlon atrial septectomy

Each of these procedures are performed through an incision between the ribs and visualized by echocardiogram; these are far less common than heart cath procedures.

Pulmonary artery banding is used in a small number of cases of d-TGA, usually when the corrective surgery needs to be delayed, to create an artificial stenosis in order to control pulmonary blood pressure; PAB involves placing a band around the pulmonary trunk, this band can then be quickly and easily adjusted when necessary.

An atrial septectomy is the surgical removal of the atrial septum; this is performed when a foramen ovale, PFO, or ASD are not present and additional shunting is required to raise the oxygen saturation of the blood.

Major

• Median sternotomy
  • PAB (when intracardiac procedures also required)
  • Concomitant atrial septectomy

In recent years, it is quite rare for palliative procedures to be done via median sternotomy. However, if a sternotomy is required for a different procedure, in most cases all procedures that are immediately required will be performed at the same time.

Total anomalus pulmonary venous return

Surgery should be performed as soon as possible in the patients of total anomalous pulmonary venous connection. The surgical procedure varies depending upon the anatomy of the TAPVC lesion.

• Supracardiac and infracardiac TAPVC: Connection is created between the pulmonary veins and the left atrium. The vertical vein is tied up so that the abnormal blood flow could be prevented.
• Pulmonary veins directly connected to the superiorvenacava: An intracardiac baffle is created that helps in transfer of blood from the right atrium, through atrial septum into the left atrium.
• Intracardiac (pulmonary vein connected to the coronary sinus): Coronary sinus is incised and connected to left atrium.
• Intracardiac (pulmonary vein opening directly into the right atrium): A interatrial connection is made and the blood is redirected from right atrium to left atrium.

Tricuspid atresia

First stage

First stage of surgery is performed in the neonatal period. The goals of the initial palliation are to ensure that blood exiting the right atrium (RA) is unimpeded, provides adequate pulmonary blood flow, protects the pulmonary artery bed from high pressures that could result in higher risk for subsequent operations, and ensures unobstructed flow from the left ventricle (LV) to the aorta. Choices for intervention are dependent on the anatomic variants as follows (table 1):

●TV atresia with normally related great arteries (type I):

•With diminished pulmonary blood flow, the initial surgery soon after birth aims to restore a reliable source of pulmonary blood flow with a modified Blalock-Taussig shunt (shunt from the innominate artery into the central pulmonary artery) (figure 4).

•In the presence of unobstructed pulmonary blood flow (type Ic), a pulmonary artery band may be placed in early infancy to restrict the amount of pulmonary blood flow and protect the pulmonary bed from high systemic pressures. As mentioned above, the vast majority of ventricular septal defects (VSDs) decrease in size over time and restrict pulmonary blood flow. A pulmonary artery band, therefore, may not be required in all patients who have unobstructed pulmonary flow at birth through a VSD, and could be restricted to patients who are symptomatic despite maximal medical therapy for pulmonary congestion/heart failure. Furthermore, a pulmonary artery band may stimulate myocardial hypertrophy that may more rapidly reduce the size of the VSD.

•Rarely, the degree of restriction to pulmonary blood flow is enough to maintain adequate oxygenation without pulmonary overcirculation and heart failure. These patients may not require surgery in the neonatal period and can be taken directly to the second stage of palliation.

●TV atresia with transposition of great arteries (type II):

•In the presence of significant subaortic obstruction with a restrictive VSD, enlargement of the VSD or a Damus-Kaye-Stansel anastomosis (anastomosis between the main pulmonary artery and ascending aorta) with a modified Blalock-Taussig shunt is the initial surgery of choice.

•If an isolated coarctation is present, it should be relieved and a pulmonary artery band may be considered to restrict pulmonary blood flow.

•Similar to type I lesions, in a few cases, the size of the VSD is large enough to maintain systemic output, in which case a pulmonary artery band alone may be adequate.

Second stage — The second palliative procedure for both type I and type II lesions is a cavopulmonary anastomosis (Glenn procedure). This stage of palliation is typically performed at three to six months of life when infants experience progressive cyanosis as they begin to outgrow their neonatal shunt. This surgery involves removal of the original shunt, and direct anastomosis of the superior vena cava to the right pulmonary artery. The Glenn procedure relies on passive venous drainage from the superior vena cava directly into the pulmonary artery. However, there is persistent systemic desaturation due to continued inferior vena cava flow into the RA. In a case series of 557 patients who underwent second stage palliation for single ventricle anatomy from 1998 to 2010, the overall mortality rate was low (4.7 percent) and there were no deaths in the 12 percent of patients who had TV atresia [25]

Tetralogy of fallot

Palliative surgery

• The surgery involved forming an anastomosis between the subclavian artery and the pulmonary artery.
• The Pott shunt and the Waterson procedure are other shunt procedures which were developed for the same purpose.
• Potts shunt: Shunt between left pulmonary artery and descending aorta.
• The technique has been modified and is usually performed using a Gortex tube to create the connection.
• Waterson shunt: Shunt between right pulmonary artery and ascending aorta.

Total Surgical Repair

• The surgery generally involves:
  • Making incisions into the heart muscle, relieving the right ventricular outflow tract stenosis by careful resection of muscle
  • Repairing the VSD using a Gore-Tex or Dacron patch or a homograft.
  • Additional reparative or reconstructive work may be done on patients as required by their particular anatomy.
  • The repair could be done by either of the approaches i.e.transatrial or transpulmonary

Coarcitation of aorta

Indications for Surgery

• There is a lack of consensus for the indications and the time for surgery. Some groups of surgeon consider balloon angioplasty and stenting as the initial approach and reserving surgery for more complicated conditions or treatment failures. Whereas, others consider surgical repair as the first line of therapy.
• Surgery is the first choice in aortic coarctations involving:
  • Long length of aorta
  • Severe coarctations causing almost complete or complete obliteration of the aortic lumen.
  • Associated with other cardiac defects for e.g. large patent ductus arteriosus, ventricular septal defect.

 Surgical Techniques

• The choice of technique depends on the patient's age at presentation, size, associated abnormalities, and anatomy of the coarctation.
• Surgical approach - median sternotomy is preferred over left lateral thoracotomy, in complex arch repairs.
• Subclavian flap aortoplasty is found to be the most commonly performed followed by resection in end-to-end anastomosis, patch aortoplasty, and bypass graft when the surgery is done during infancy. Whereas, in children and adolescent undergoing coarctation repair end-to-end anastomosis is commonest followed patch aortoplasty and subclavian flap aortoplasty.
• Patch aortoplasty are less frequently used these days because of concerns regarding the development of aortic aneurysm at the site of surgery. 
• Available Techniques:
  • Resection and end-to-end anastomosis
  • Patch aortoplasty
  • Left subclavian flap aortoplasty
  • Tubular bypass grafts
  • Combination techniques 

Atrial septal defect

• Surgical closure is the most common method of treatment method for atrial septal defect and has been the gold standard for many years. Many surgeons prefer more minimally invasive techniques over the conventional sternotomy to avoid potentials for additional complications. Special consideration must be taken into account for the age of the patient and the size of the defect involved. Surgical closure is indicated for patients with primum, sinus venosus and coronary sinus type of atrial septal defects. However, ostium secundum atrial septal defects are commonly treated by percutaneous closure. With uncomplicated atrial septal defect, (without pulmonary hypertension and other comorbidities) the post-surgical mortality is as low as 1%. Minimally invasive repair of atrial septal defect has been shown to be as successful as the conventional sternotomy. Although they have not been associated with reduced morbidity and mortality rates, they have been proven to have the advantage of being less invasive, less post-surgical complications, decreased hospital stay, and more cosmetic benefits.

Pulmonary hypertension

• The choice of treatment for pulmonary hypertension requires the assessment of the clinical severity of the disease and the identification of any underlying cause.
• Patients who have PH secondary to a medical condition such as left heart failure, lung diseases, or thromboembolic disease should receive treatment for the underlying cause.
• Patients who have pulmonary arterial hypertension (PAH) must undergo vasoreactivity testing in order to assist in the selection of the optimal therapy which includes calcium channel blockers, endothelin receptor antagonist, phosphodiesterase inhibitors, or prostanoids.
• Surgical intervention such as atrial septostomy or lung transplantation should be considered among patients with pulmonary arterial hypertension who fail to improve on optimal therapy or when medical therapy is unavailable.
• Failure of clinical improvement among PAH patients with WHO functional class II or III is defined as either:
  • A stable and unsatisfactory clinical status, or
  • An unstable and deteriorating Failure of clinical improvement among PAH patients with WHO functional class IV is defined as either:
  • Absence of quick improvement to a WHO functional class III or less, or
  • A stable and unsatisfactory clinical status

Atrial Septostomy

• Atrial septostomy is a surgical procedure that creates a shunt between the right and left atria.
• It relieves pressure on the right side of the heart, but at the cost of lower oxygen levels in blood (hypoxia). It is best performed in experienced centers.
• Graded balloon dilatation of atrial septostomy is the recommended type of atrial septostomy.
• Atrial septostomy might be considered among PH patients with WHO functional class IV and right heart failure refractory to therapy.
• Atrial septostomy should also be considered among PH patients who have Eisenmenger's syndrome, idiopathic PAH, and those awaiting lung transplantation.

Lung Transplantation

• Lung transplantation is considered in the treatment of patients with idiopathic PH, PH associated with congenital heart disease, or pulmonary veno-occlusive disease (PVOD) who fail to improve on optimal medical therapy.
• Combined lung and heart transplantation might be considered in selected patients.
• According to the Registry of the International Society for Heart and Lung Transplantation, the survival rates following lung transplantation are 61%, 49%, and 25 % at 3, 5, and 10 years respectively.

Pulmonary Thromboendarterectomy

• Pulmonary thromboendarterectomy (PTE) is a surgical procedure that is used for the treatment of chronic thromboembolic pulmonary hypertension.
• It is the surgical removal of an organized thrombus along with the lining of the pulmonary artery.
• PTE is a large and very difficult procedure that is currently performed in a few select centers. Case series show remarkable success in most patients.
• Treatment for hypoxic and miscellaneous varieties of PH have not been established. However, studies of several agents are currently enrolling patients. Many physicians will treat these diseases with the same medications as for PAH, until better options become available.