Glossary

Congenital heart disease – An introductory glossary of terms

Anomalous pulmonary venous drainage:

partial – at least one, but not all, pulmonary veins connect to the right heart, often via the superior or inferior vena cava, leading to varying degrees of left-to-right shunt (Qp:Qs>1). Total – all pulmonary veins abnormally drain to the heart. Types include: supracardiac – where they eventually drain to the SVC, intracardiac – where they drain through the coronary sinus, and infracardiac – where they drain beneath the diaphragm to the portal venous system.

Aortopulmonary window: a window (communication) between aorta and main or right pulmonary artery. May be associated with interrupted aortic arch. 

Atrial septal defect: types include patent foramen ovale (PFO), secundum, primum (a type of AV defect), sinus venosus (superior: at SVC to RA junction, associated with PAPVD of right upper and middle PVs; inferior: at IVC to RA junction, associated with PAPVD right lower PV), coronary sinus (unroofed coronary sinus leading to communication between left atrium and coronary sinus). Leads to left-to-right shunt. 

AVSD: atrio-ventricular septal defect due to incomplete fusion of the endocardial cushions, leading to malformation of the mitral and tricuspid valves, atrial and ventricular septal defects. A common atrioventricular valve straddles the ventricular septum.

Balanced circulation: equal pulmonary and systemic blood flow (Qp:Qs = 1).

Bidirectional Glenn/bidirectional cavopulmonary shunt/superior cavopulmonary shunt (SCPC)/hemi-Fontan: SVC disconnected from RA and anastomosed to PA, causing pulmonary blood flow to comprise SVC venous blood. It is bidirectional because SVC blood is free to flow through either right or left PA (in contrast to aunidirectional/original Glenn shunt where the SVC was directly anastomosed to the right PA that had been disconnected from the main PA, thereby preventing SVC blood from flowing to the left PA). 

Blalock-Taussig (-Thomas) shunt (BT shunt): original – transection of subclavian artery and end-to-side anastomosis to ipsilateral pulmonary artery. Modified – a graft conduit placed between either the innominate or subclavian artery and the ipsilateral pulmonary artery. Designed to allow some pulmonary blood flow through a systemic-to-pulmonary connection.

Brom repair: a 3-patch technique that allows repair of supra-aortic narrowing with enlargement of all 3 sinuses.

Carpentier’s procedure: a method for tricuspid valve repair of Ebstein’s anomaly, characterised by mobilisation of anterior leaflet, vertical plication of the atrialised RV, advancement of the anterior leaflet across the plicated area to reduce orifice size, and insertion of an annular ring for additional valve strength.

Central shunt: small tubular connection created between ascending aorta and main pulmonary artery.  Designed to provide some pulmonary blood flow through a systemic-to-pulmonary connection. 

Coarctation of the aorta: a narrowing of the thoracic aorta, usually distal to the left subclavian artery. Associated with bicuspid aortic valve.

Cone procedure/reconstruction: a method for tricuspid valve repair of Ebstein’s anomaly, characterised by mobilisation of valve leaflets, maximisation of leaflet tissue through delamination of the valve tissue from the myocardium whilst maintaining chordae attachment, rotation and suturing of the leaflet complex to create a cone with base at the TV annulus and vertex in the right ventricular apex, and plication of atrialised RV.

Cor triatriatum: a restrictive membrane in the left atrium, dividing the atrium into two chambers. Blood from the upper chamber, where the pulmonary veins enter, drains into the lower chamber through one or more orifices in the membrane, with symptoms dependent on the degree of restriction through the membrane.

Damus-Kaye-Stansel operation: a proximal PA to aorta anastomosis (above their respective valves). It provides systemic flow in situations where there is LVOT obstruction or subaortic stenosis in functional univertricular hearts. Usually combined with either a BT shunt or a RV-PA conduit, which supplies pulmonary blood flow.

Double inlet left ventricle: both atria drain into a single ventricle, which is commonly connected to an additional hypoplastic ventricle via a bulboventricular foramen. The most common subtype is a double-inlet LV, which is anatomically right-sided, and has ventricular-arterial discordance (TGA).

Double outlet right ventricle: a defect in which both pulmonary artery and aorta arise from the right ventricle. A VSD is present, positioned either in a sub-pulmonary, sub-aortic, doubly-committed, or remote location. The resultant physiology can mimic that of tetralogy of Fallot, transposition of the great arteries, or a VSD, depending on the relation between the position of the VSD to the great arteries.

Ebstein’s anomaly: abnormality of the tricuspid valve resulting in abnormal downward and rotational displacement of the valve orifice into the RV, with atrialised portion of the RV between the annulus and abnormal leaflet attachments.

Fontan operation/Fontan completion/total cavopulmonary connection (TCPC): final stage in univentricular palliation (after a previous Glenn/SCPC/hemi-Fontan procedure. The IVC is connected to the PA in one of 3 ways; originally through a RA-PA direct anastomosis, or through a lateral tunnel within the RA, or through an extra-cardiac conduit (now most common approach). A fenestration between the IVC and RA may be created to provide a right-to-left ‘pop-off’ pathway from the cavopulmonary circuit to the heart in order to lower the pressure in the circuit, albeit at the expense of lower saturations. 

Heterotaxy: a complex syndrome of malformation, due to abnormal sidedness of thoracic and abdominal organs. Potential abnormalities include atrial or lung isomerism, single ventricle, poly- or a-splenia, bilateral or persistent left SVC, TAPVD, left-sided or interrupted IVC, and intestinal malrotation.

Holmes heart: a specific sub-type of DILV, with a hypoplastic morphological right ventricle, and normally related great arteries.

Interrupted aortic arch: interrupted or atresic arch, leading to complete distal disruption or obstruction to flow.

Jatene procedure: this procedure is an arterial switch operation and is a definitive treatment for transposition of the great arteries. Both the aorta and pulmonary arteries are transected. The aorta is anastomosed to the residual proximal pulmonary artery, which becomes the neo-aorta. The distal pulmonary artery is anastomosed to the residual proximal aorta. Commonly a Lecompte manoeuvre is carried out during this step. Crucial to the success of the procedure is the successful transfer of the coronary origins from the native aorta to the neo-aorta.  

Konno procedure: in order to relieve LVOT obstruction associated with aortic valve annulus hypoplasia, the aortic valve is excised and an incision in the ventricular septum is made and patched open. The creation of a widened LVOT allows a larger aortic valve to be used. This can be with either a mechanical (Konno-Rastan), homograft, or autograft (Ross-Konno) replacement. 

Lecompte manoeuvre:  the distal main pulmonary artery is brought anterior to the aorta so that the left and right PA bifurcation straddles the anterior ascending aorta. 

MAPCAs: major aorto-pulmonary collateral arteries. These vessels are likely dilated bronchial arteries and provide a degree of pulmonary blood flow. They can provide a portion or all pulmonary blood flow depending on the degree of pulmonary arterial flow, or presence (or absence) of alternative conduits for pulmonary blood flow (e.g. PDA, BT shunt). They are prone to stenosis.

Mustard procedure: an atrial switch procedure for patients with transposition of the great vessels that creates an intra-atrial baffle made of native pericardium or synthetic material. This redirects pulmonary venous blood through the tricuspid valve into the right ventricle, and systemic venous blood through the mitral valve into the left ventricle. By surgically creating atrio-ventriclular discordance, physiologic (but not anatomical) correction is achieved.

Norwood procedure: first of a three stage palliative process for patients with univentricular hearts. It involves transection of the pulmonary artery, amalgamation of the proximal pulmonary artery with aorta, aortic arch augmentation, atrial septectomy, and provision of pulmonary blood flow either through a systemic-to-pulmonary artery shunt (BT shunt), or via an RV-PA conduit (Sano shunt/modification).

Patent ductus arteriosus: Persistence of a patent ductus arteriosus results in left-to-right shunt from aorta to pulmonary artery, and can lead to pulmonary overcirculation, pulmonary hypertension and heart failure.

Potts shunt: fenestration-type direct communication created between descending aorta and left pulmonary artery. Designed to provide some pulmonary blood flow through a systemic-to-pulmonary communication.

Rastelli procedure: in situations such as certain sub-types of double outlet right ventricle, LV outflow is accomplished by routing blood across a VSD patch, which serves as a baffle. An RV-PA conduit provides pulmonary blood flow.

Ross procedure: for some cases of aortic valve replacement, the patient’s own pulmonary valve (pulmonary autograft) is transposed to the aortic position. A cadaveric or bioprosthetic pulmonary valve is then used to replace the patient’s native pulmonary valve. The advantage of this procedure is the superior longevity of an autograft compared with currently available homografts, and is the only option that provides the possibility of growth, thereby appealing for use in children. 

Sano shunt: an RV-PA conduit.

Senning procedure: a similar procedure to the Mustard procedure, although autologous tissue from the right atrial wall or inter-atrial septum is used rather than the pericardium or synthetic material used in the Mustard procedure.

Starnes procedure: as an alternative to biventricular repair of Ebstein’s anomaly, the Starnes procedure involves patch closure of the TV, reduction atrioplasty and/or RV plication, formation of an unrestrictive ASD, and creation of pulmonary blood flow through a mBT shunt, thereby creating single ventricle physiology.

Taussig-Bing anomaly: a heart with a double outlet right ventricle with a subpulmonary VSD. Left ventricular oxygenated blood is preferentially streamed into the pulmonary artery, and therefore behaves physiologically as a heart with transposition of the great arteries. 

Transposition of the great arteries: in d-transposition, there is ventricular-arterial discordance, whereby the aorta arises from the right ventricle and the pulmonary artery arises from the left ventricle. Treatment historically involved either a Mustard or Senning procedure. Current definitive treatment is a Jatene procedure (arterial switch). In the rarer l-transposition (synonymous with ‘congenitally-corrected transposition’), there is both atrio-ventricular and ventricular-arterial discordance. The right atrium drains blood through a mitral valve into a right-sided morphological left ventricle, which ejects into the pulmonary artery. The left atrium drains blood through a tricuspid valve into a left-sided morphological right ventricle, which ejects into the aorta. Treatment may include a double switch, involving both a Senning and a Jatene procedure, to allow the morphological left ventricle to become the systemic pump. 

Truncus Arteriosus: a defect in which the pulmonary artery and aorta exit the heart as a single trunk. There is a VSD, which the trunk overrides.

Ventricular septal defect: a defect in the ventricular septum. When large and unrestricted, this commonly leads to significant left-to-right shunting and pulmonary overcirculation. A number of types exist depending on position within the septum and relation to intracardiac structures, including inlet, outlet, perimembranous, muscular, and apical.

Waterston shunt:  fenestration-type direct communication created between ascending aorta and right pulmonary artery. Designed to provide some pulmonary blood flow through a systemic-to-pulmonary communication.

Congenital references:

Andropoulos D, et al. Anaesthesia for congenital heart disease. 3^rd edition. 2010. Wiley: USA.

Barry P, Morris K, Ali T. Paediatric intensive care (oxford specialist handbook). 2010. Oxford University Press: UK.

Everett A, Lim D. Illustrated field guide to congenital heart disease and repair. 3^rd edition. 2015. Scientific Software Solutions, Inc: USA.

Lake C, Booker P. Pediatric cardiac anesthesia. 4^th edition. 2004 Lippincott Williams and Wilkins: London.