Acute Rheumatic Fever (ARF) is common in Samoa, Tokelau, Tonga, Fiji and the Pacific Region. It affects mostly children between the ages of 5 and 15 years old, with possible devastating long-lasting effects on the heart. 

ARF is started by the common nasty germ called Group A Streptococcus (GAS) that invades and infects the throat causing a sore throat, and also infects the skin causing impetigo in children or “po’u” in Samoa. As the body mounts an attack against the GAS in the throat or the skin, this body reaction can cause symptoms and affect the heart (carditis - causing leaky heart valves), joints (arthritis - swollen or painful joints), skin (red rash – erythema marginatum), subcutaneous nodules under the skin, and can affect the brain (chorea – involuntary movement of the hand or foot).

All these complications of ARF do not last for a long time, except for the effect on the heart called Rheumatic Heart Disease (RHD), which can persist beyond 30 or 40 years old or persist for life. The heart can become quite weak (called heart failure) due to the severe leakiness of the valves. Many with ARF do not have symptoms. 

In Samoa, many of our young people have died from severe RHD, and we have sent many young people to New Zealand every year for major life-saving heart operations that cost NZD$60,000 per operation under the Samoa Government Overseas Treatment Scheme. Samoa is one of a handful of countries in the Pacific that funds the treatment of their people in New Zealand or India. Last year, the Government of Samoa spent about SAT$16 million for overseas treatment, and much of this was for RHD. 

ARF is preventable, and GAS sore throat or impetigo (po’u) can be treated with Penicillin to stop the development of ARF. To prevent further attacks of ARF and to prevent the development and progression of RHD, we need to administer monthly or 4-weekly intramuscular (IM) Penicillin injections. The Samoa health system is well set up to perform all these needed IM Penicillin injections in all the hospitals. Severe RHD kills many young people, therefore we have to pick these up early so that we can save the children. It is unfortunate that many ARF and RHD have no symptoms at all, and therefore Echo Screening is one of the best ways to pick up RHD early.

The National Echo RHD Screening Program in Schools in Samoa was started by heart specialist (cardiologist) Professor Asiata Dr Satupaitea Viali from 2008 until 2018, to try to manage the massive burden of RHD in Samoa. Samoa was the first country in the Pacific and one of the first countries in the world to start its National Echo Screening Programs. Just before the screening program in Samoa started in early 2008, the French (Cambodia and Mozambique) and South America (Nicaragua) started their screening programs at the end of 2007 which were published in major medical journals. The rest of the world followed suit in South Africa, India, Australia, New Zealand, Fiji and Tonga in 2012-2014.

The World Heart Federation (WHF) selected a team of cardiologists and paediatricians from around the world in 2009 who worked with ARF/RHD to research and create criteria for diagnosing RHD using the Echo Scan. Prof. Satu was part of this WHF Expert Group. This research work was published in 2012 in Nature Cardiology Journal. Every Screening Research in all countries uses the standards developed by the WHF Expert Group in 2012. These guidelines have been updated in 2023 and published in September 2023 in Nature Cardiology. The Echo is a special scan of the heart that uses ultrasound waves to view the inside of the heart and its valves, which is a lot more sensitive than using the stethoscope. The Echo also gives the structural integrity of the heart and valves, and if there is any leak or stenosis of the valves, it can view and calculate the degree of severity.

Prof. Satu has done 30,000 Screening Echos in Primary Schools and Secondary Schools in Samoa in Upolu and Savai'i from 2008 to 2018. He had done all the primary schools and colleges in Apia and the rural regions. Another team from America with many Echo sonographers and a cardiologist, have screened another 30,000 kids in primary schools only, in Upolu and Savai'i. Many of the kids who were picked up with RHD have been treated with 4-weekly IM Penicillin in the various hospitals in Savai'i and Upolu. The Echo follow-up of these kids picked up from school had been ongoing for many years and we have seen a significant number of RHD regression. 

The follow-up of these young people on IM Penicillin over the years has been challenging. Those with congenital heart disease were also treated and some with severe heart diseases (severe ASD, severe VSD) were sent for surgery. Since the COVID-19 lockdown, the National Echo Screening Program has not started again. Prof. Satu will be travelling around the Pacific Region to do the Echo Screening work in other countries that need this work done. RHD Screening is one of his sub-specialties in cardiology.

In August this year, the Tokelau Ministry of Health contracted Prof. Satu to perform a National Echo Screening Program on the three atolls of Tokelau. Though there have been small numbers (only 5) of ARF and RHD in Tokelau, the Taupulega and the Minister of Health of Tokelau have requested for Prof. Satu to do the Echo Screening Program and Cardiology Clinics on the Island. Tokelau is a dependent territory of New Zealand in the southern Pacific Ocean. It consists of three tropical coral atolls (Atafu, Nukunonu and Fakaofo) with a combined land area of 12 km²  (4 sq miles). At roughly 8 km (5 miles) wide, Atafu Atoll is the smallest of three atolls comprising Tokelau. The land area is only about 2.5 km² and the lagoon's area is about 15 km². The primary settlement on Atafu is a village located at the northwestern corner of the atoll - indicated by an area of light grey dots in the left part of this photograph.

The typical ring shape of the atoll is the result of coral reefs building up around a former volcanic island. Over geologic time, the central volcano subsided beneath the water surface, leaving the fringing reefs and a central lagoon that contains submerged coral reefs. Nukunonu is the largest of the three islands that comprise Tokelau. It is composed of a roughly tear-drop-shaped atoll that includes 5.5 km² of land and whose central lagoon encompasses an area of about 90 km². Fakaofo is one of the three islands that make up Tokelau. Its land area is only 3 km², the lagoon covers an area of about 45 km². The total Tokelau population is 1,500 in 2023 in the three atolls, with a GDP of USD$7,445 per capita, which is much better than Samoa’s GDP of USD$4,265 per capita with a total population of 202,093. Fakaofo sits roughly 500 km north of Samoa. Nukunonu lies 64 kilometres north-west of Fakaofo, and Atafu lies 92 kilometres north-west of Nukunonu. The three atolls have four villages, with Nukunonu and Atafu having one each, and Fakaofo having two. Tokelau has close ties to Samoa, with many Tokelau people living in Samoa. Boats transporting passengers and cargo arrive from Samoa approximately once a week. The only way to reach Tokelau is by boat, which takes a minimum of 24 hours to reach the first atoll. 

Over the 15 days (3 weeks) of the screening clinics, a total of 638 Echo Scans were done in Nukunonu, Atafu and Fakaofo, which equates to about 43 per cent of the total population, doing 43 Echo Scans per day or 5-6 Echos per hour. Everyone between the ages of 4 years old and 20 years old was screened and 94 per cent of this population was screened for heart disease, especially RHD. There was usually one primary and secondary school in each atoll plus the USP extension courses for the older adults. The directions and approval to do this National Echo Screening work were given by the Taupulega, the highest council of each atoll. The people were given the choice to participate.

Prof. Satu attended the Taupulega Council in each atoll before the screening work started, where he was blessed by the elders to do the work. For the screening work of age 4-20 years old, every person was auscultated with the stethoscope to determine any heart murmur before the Echo Screening, as auscultation determined the length of the Echo Scan. The older folks above age 20 years old were invited by the Taupulega to have a “cardiac check”. 

"I was privileged to do the full cardiac checks for the known cardiac patients in Tokelau utilising my clinical skills, ECG, and Echo procedure to diagnose and manage their heart diseases," said Prof. Satu. "The adults who needed screening for heart diseases had a brief history taken, clinical examination, ECG and Echo done. Many 'normal' older adults had RHD without knowing they had anything wrong with them."

The National Echo Screening Program in Tokelau had identified a number of ARF and RHD in the younger and older people (age range 5–50 years old) of Tokelau, that needed treatment and follow-up. All those people below the age of 30 years old have now been treated with 4-weekly IM penicillin and their prognosis is very good with treatment. Only one older person with significant RHD will need a major heart valve operation in New Zealand over the next 12 months. The situation in Samoa is similar to Tokelau.

The National Echo Screening Program had assisted the Department of Health in Tokelau in defining the burden of RHD. Fortunately, each hospital in each atoll can treat its own ARF and RHDs. The Medical Officers and staff nurses and the health system in Tokelau are well trained and equipped for treatment and prophylaxis of ARF and RHD, with intermittent input from Prof. Satu if needed, over the last 10 years. The next stage is teaching the staff to screen with hand-held Echo machines, so that, they can do their own screenings, and the abnormal cases are referred to the cardiologist Prof. Satu for checking and confirming. This training is in the planning stages and is most likely to be conducted by Prof. Satu. 

The Taupulega of Nukunonu Atafu and Fakaofo, Minister of Health, Department of Health and the people of Tokelau were very appreciative of this screening work. This work will be repeated again in 3-4 years’ time or whenever the Taupulega requests. 

• Editor's Note: Professor Asiata Dr Satupaitea Viali, a Specialist Physician and Cardiologist in Samoa, contributed to and provided editorial guidance for this analytical piece.