The Fontan epidemic: population projections from the Australia and New Zealand Fontan registry

Background: The number and age demographic of the future Fontan population is unknown

Hospital inpatient costs for single ventricle patients surviving the Fontan procedure

We estimated the inpatient resource use for a Fontan patient from birth to adulthood and explored factors that might induce cost differences (2014 US dollar). Inpatient costing records from 4 hospitals with greatest numbers of Fontan patients in Australia and New Zealand were linked with the Fontan registry database. Inpatient records between July 1995 and September 2014 for 420 Fontan patients were linked, and the most frequent primary diagnoses were hypoplastic left heart syndrome (20.7%), tricuspid atresia (19.7%), and double inlet left ventricle (17.1%). The mean hospital cost for a Fontan patient from birth to 18 years of age was estimated to be $390,601 (95$264,703 to $516,499), corresponding to 164 (95$219,482 (95% CI $202,410 to$236,553) and the cost thereafter over 15 years was $146,820 (95$44,409 to $249,231), corresponding to 82 (95% CI 72 to 92) and 65 (95% CI 18 to 112) inpatient days, respectively. Costs were higher in male and hypoplastic left heart syndrome patients in the staged procedures period (

Hepatic and renal end-organ damage in the Fontan circulation: a report from the Australian and New Zealand Fontan Registry

Background: Hepatic and renal dysfunction have been observed in survivors of the Fontan procedure, however their incidence and associated factors remain poorly defined. Methods: A total of 152 participants from a Registry of 1528 patients underwent abdominal ultrasound, transient elastography (FibroScan), serum fibrosis score (FibroTest), in vivo Tc-99m DTPA measurement of glomerular filtration rate (mGFR), and urine albumin-creatinine ratio (ACR). Results: Mean age and time since Fontan were 19.8 ± 9.3 and 14.1 ± 7.6 years, respectively. Features suggestive of hepatic fibrosis were observed on ultrasound in 87/143 (61%) and no patient was diagnosed with hepatocellular carcinoma. FibroScan median kPa was ≥10 in 117/133 (88%), ≥15 in 75/133 (56%), and ≥20 in 41/133 (31%). Fifty-four patients (54/118, 46%) had a FibroTest score ≥0.49 (equivalent to ≥F2 fibrosis). FibroTest score correlated with FibroScan value (r = 0.24, p = 0.015) and ACR (r = 0.29, p = 0.002), and patients with ultrasound features of hepatic fibrosis had a higher FibroScan median kPa (19.5 vs 15.4, p = 0.002). Renal impairment was mild (mGFR 60–89 ml/min/1.73 m) in 46/131 (35%) and moderate (mGFR 30–59 ml/min/1.73 m) in 3/131 (2%). Microalbuminuria was detected in 52/139 participants (37%). By multivariable analysis, time since Fontan was associated with increased FibroScan median kPa (β = 0.89, 95% CI 0.54–1.25, p = 0.002) and decreased mGFR (β = −0.77, 95% CI −1.29–0.24, p = 0.005). Conclusions: In the second decade after Fontan hepatic and renal structure and function are abnormal in a significant number of patients: close to 60% have ultrasonographic evidence of structural hepatic abnormalities, 46% have elevated serum hepatic fibrosis scores, and 57% have either reduced glomerular filtration rate or microalbuminuria. Hepatic and renal function should be monitored for potential impacts on outcomes after Fontan completion

Sick Sinus Syndrome: Experience of a Cardiac Pacemaker Clinic

Out of a pacemaker clinic population of 182 patients 21 (11·5%) were found to have the sick sinus syndrome. Their ages ranged from 30 to 80 years and averaged 62. Ischaemic heart disease was more commonly an aetiological factor than in patients with chronic atrioventricular heart block. Other aetiologies were familial cardiomyopathy, postcardiac surgery, and dystrophia myotonica. Cardioversion before pacemaker insertion was a hazardous procedure. After insertion the occurrence of tachycardias and the need for drug therapy were reduced. One patient no longer required a pacemaker once atrial fibrillation became established. A high incidence of cerebral embolization was observed and the use of anticoagulant drugs therefore merits serious consideration. Failure of inhibition of demand-type pacemakers occurred in two patients. Two patients who exhibited competition with fixed-rate pacemakers died. Two patients were treated with electrodes surgically implanted on the right atrium. It is suggested that fixed-rate pacemakers may be contra-indicated and that sequential atrioventricular demand pacing is theoretically ideal

Oral Contraceptives and Myocardial Infarction

Between January 1970 and December 1972 22 women aged between 31 and 45 years were admitted to the coronary care unit with acute myocardial infarction and six of these (27%) had been taking oral contraceptives. There were nine women aged 40 or less and five of them (55%) had been on oral contraceptives while three of the other four had been sterilized by tubal interruption. Both these figures of prevalence of oral contraceptive use are significantly greater than estimates for the general population of women of similar age. For those aged 30-44 years, current estimates suggest that it is between 8 and 11%. All the women in this study had risk factors recognized as being associated with the premature development of ischaemic heart disease, and the prevalence of these risk factors was similar in those taking oral contraceptives as in those not doing so. Oral contraceptives probably enhance the chance of developing myocardial infarction in women whose risk is increased for other reasons

Treatment of haemoptysis in pulmonary atresia with tranexamic acid

We report the case of a young woman with continuing haemoptysis, pulmonary atresia, previous shunt surgery, and pulmonary hypertension. She was not suitable for further surgery or for therapeutic embolisation of bronchial vessels. Treatment with tranexamic acid resolved the haemoptysis

A qualitative study of adversity activated development and resilience in adolescents and young adults with CHD and their parents

The majority of infants born today with heart defects will survive to adulthood. Life for adults with Congenital Heart Disease (CHD) may appear 'normal', but be punctuated by illness-related events such as hearing a 'bad' prognosis, experiencing uncontrollable arrhythmias, or having complicated heart surgery. Therefore living with CHD can be traumatic. However, it is possible to develop attributes in response to trauma that enable not just survival, but also self-improvement. This important concept has been termed Post-Traumatic Growth (PTG) or Adversity Activated Development (AAD). We will adopt AAD terminology to emphasise the developmental component of coping and recovery. Based on a thematic analysi of 10 CHD patients and 10 parents, our study participants described positive, neutral and negative personal consequences to living with CHD. The results indicate that achievement of AAD may be related to having acceptance, a sense of coherence (or a belief that obstacles can be managed and that life is meaningful with lessons to be learned), and a sense of connectedness (special relationships with peers, family and medical staff). These findings highlight psychological processes to facilitate in order to maximise AAD in young people with CHD.</p

Coronary stenting for a muscular bridge in a patient with hypertrophic obstructive cardiomyopathy

A young woman with hypertrophic cardiomyopathy presented with intractable chest pain due to a myocardial bridge over the left anterior descending artery, producing severe compression during systole. Percutaneous intracoronary stenting was performed. The patient, however, developed severe and diffuse restenosis within 30 days of the procedure. Myotomy may provide a more effective treatment option for such patients with symptomatic myocardial bridging