You are reviewing a 27-year-old male in clinic for the first time. On a routine health check 12 months previously he was found to have a restrictive perimembranous VSD on his echocardiogram. The jet velocity was measured at 5 m/s. There was no evidence of left ventricular dilatation and pulmonary pressures are not raised. He is asymptomatic. The rest of his echocardiogram confirmed a structurally normal heart apart from mild aortic regurgitation. You repeat the echocardiogram in clinic and there has been no change.
What is the most appropriate follow-up?
Restrictive VSDs, by definition, have no haemodynamic consequences. There is an increased risk of endocarditis but no role for prophylactic antibiotics based on current guidance. They can cause aortic valve prolapse (usually right coronary cusp) and progressive dysfunction as a result of the Venturi effect of the high-velocity jet and turbulence below the AV. Patients with evidence of any degree of AV regurgitation require close follow-up for progression as surgical repair of the VSD is indicated prior to irreversible valve damage.
You are asked to review and explain the terminology on an echocardiogram report for a patient who has just returned to the ward having been admitted with stable but symptomatic AV block. The report states that there is A–V and V–A discordance.
What is the underlying diagnosis?
The terms are based on the sequential segmental approach of describing anatomy based on the cardiac component and connections from atria to ventricles (A–V) and ventricles to great vessels (V–A). A–V and V–A discordance describes ccTGA where the ventricles are inverted. If the RA connects to the morphological LV (through the ‘mitral’ valve)
Ao, aorta; LA, left atrium; LV, left ventricle; PA, pulmonary artery; MV, mitral valve; RA, right atrium; RV, right ventricle; TV, tricuspid valve.
and the LA connects to the morphological RV (through the ‘tricuspid’ valve), this is A–V discordance. If the LV then connects to the PA and the RV connects to the aorta, this is V–A discordance. AV block is common in patients with ccTGA and may be the presenting complaint in an undiagnosed adult. The other common presentation is heart failure, as the RV and TV are not designed for systemic work and eventually ‘wear out’. In TGA there is A–V concordance but V–A discordance
You are asked to review a GUCH patient at 03:00 who has directly attended the ED with palpitations and breathlessness. On his arrival, the notes are available and document a diagnosis of tricuspid atresia with Fontan surgery. The patient appears anxious but well and tells you the symptoms started 8 hours previously whilst he was eating. He is well perfused with heart rate 130 bpm, BP 110/70 mmHg, and saturations of 97% on room air. There are no clinical signs of heart failure. The ECG is shown below.
The QRS morphology is consistent with baseline ECGs. The patient is on warfarin with an INR of 2.7.
What is your management strategy?
Tachyarrhythmias in patients with Fontan circulation is a medical emergency. Although they can appear well, there is a risk of rapid decompensation. These patients depend on LA contraction and effective left-sided diastolic haemodynamics to maintain pulmonary flow. Dehydration and arrhythmia can be fatal and prompt return of sinus rhythm is paramount.
You receive a letter from a GP asking if a patient requires follow-up in clinic. She is 35 years old and has not been seen since discharge from the paediatric cardiology services. She had a coarctation repair in childhood with no associated lesions. You have the surgical information, which documents a Dacron patch aortoplasty technique with excellent result and no residual stenosis. She is otherwise well with BP 120/80 mmHg. She has normal peripheral pulses and no murmurs. She has had two successful pregnancies. What should you advise the GP?
The Dacron patch aortoplasty technique has been shown to be associated with a risk of late aneurysm formation. Therefore there may not be any evidence of re-coarctation but a risk of aneurysm in this patient. MRI will be the best follow-up modality as it will provide structural and physiological data without radiation. The brain should also be scanned to look for berry aneurysms. If a patient has had a coarctation stent, MRI does not have the resolution of CT in detecting stent fracture and the latter may be preferable depending on the situation. The other common surgical techniques for coarctation repair are end-to-end anastomosis and subclavian flap repair (left sublavian artery is used to augment coarctation site). These can be associated with re-coarctation or pseudo-aneurysm and require long-term follow-up for BP control and the possibility of transcatheter stenting.
A 33-year-old male has been admitted under the stroke physicians with an episode of transient left upper limb weakness, which lasted 1 hour after exercising at the gym. He has no prior medical history. He is a lifelong non-smoker with no important family history. He is very fit and plays competitive basketball. Blood tests reveal total cholesterol of 4.3 mmol/L. BP is 110/70 mmHg and ECG shows sinus rhythm with normal morphology. The stroke physicians arrange a CT head and echocardiogram. The CT head returns normal. You are asked to comment on the echocardiogram report which documents a structurally normal heart with no thrombus in the LA. The only finding is of an ‘aneurysmal’ intra-atrial septum.
What should you advise the stroke team?
The case describes a young patient without any risk factors for cardiovascular disease who clinically has had a TIA. In these patients a paradoxical embolus via a PFO should be considered. ‘Aneurysmal’ intra-atrial septum describes an excessively mobile septum (septal excursion ≥10 mm with a base diameter ≥15 mm on echo). Aneurysmal intra-atrial septums commonly have associated PFO or fenestrations, and so the possibility of a communication should be suspected if seen on echo. PFO with an aneurysmal septum confers a higher risk of stroke then PFO alone. A well-performed bubble contrast echocardiogram (with sniff and Valsalva) is the investigation of choice to confirm the presence of a right-to-left shunt at atrial level.
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