Mild Pulmonary Arterial Hypertension and the Pulmonary Second Sound

Case Scenario

Case 1

A 40 year old male consulted me twice for central chest pain at rest, first during 2006 and again during 2008. The chest pain was triggered particularly by stressful work scenarios, when it would continue non-stop for a week. He furthermore suffered from impaired glucose tolerance. The pain is heavy and pressing in nature. Cardiologists performed an angiogram twice confirming that the coronary arteries were within normal parameters. His blood pressure was respectively 134/70 mmHg lying, and 102/ 60 mmHg standing. Auscultation revealed a persistent split of the second sound during an extended expiration. I diagnosed mild pulmonary arterial hypertension in a mild atopic patient.

Case 2

The above patient’s 11 year old son complained of sharp and pressing left sided chest pain accompanied by nausea, while participating in swimming competitions. The pain compelled him to stop swimming, following which the pain disappeared. The pain was not accompanied by palpitations, dizziness or faintness. On 30/10/2008 his blood pressure was respectively 113/55 mmHg lying and 92/40 mmHg standing. Cardiovascular examination revealed a left sternal heave. Auscultation revealed that the pulmonary sounds in the pulmonary area were louder than the corresponding aortic sounds; a constant split of the second sound, which varied in width during normal breathing was present. Significantly he also had an abnormal physiological split of the second sound at the apex. 

I referred him to a paediatric cardiologist who found nothing wrong during his investigations, however, he diagnosed him suffering from growing pains of the chest wall. Importantly, the colleague did not auscultate the pulmonary area during expiration in the sitting or standing position. The parents, on their own accord, took him for a second opinion to another paediatric cardiologist who came to the same conclusion after detailed investigations of what she deemed atypical chest pain. She found the second heart sounds normal with only a physiological split and a “physiological” tricuspid regurgitation. However, she also did not perform auscultation during an extended expiration in the pulmonary area sitting or standing. This colleague diagnosed reflux oesophagitis. This last diagnosis was dismissed by a gastroenterologist on the strength of the history and after gastroscopy. After the treatment which I prescribed for him he became pain free while exerting himself to the same degree which previously would have triggered his chest pain. On 08/09/2009 his blood pressure was respectively 114/50 mmHg lying and 100/45 mmHg standing. By this time the constant split of the second sound changed to a persistent split only during expiration; but last-mentioned was still clearly present.

What is it?

Pulmonary arterial hypertension (PAH) is deemed an incurable condition. This is particularly so in the case of the extreme variety of the disease. A Widlitz and RJ Barst (Text) stated the following:

“For physicians to admit that a group of patients remains for whom no cure is available in modern medicine is intellectually unsatisfying. Pulmonary arterial hypertension is a rare condition. Because the symptoms are nonspecific and the physical finding can be subtle, the disease is often diagnosed in its later stages. The natural history of pulmonary arterial hypertension is usually progressive and fatal.”  

I concur wholeheartedly with most of the above; however, it is not a rare condition. We all miss the mild variety. I too regrettably missed it regularly.

How is it Missed?

  1. It is missed due to our undergraduate training which taught us that a physiological split of the second sound means that there is not anything seriously wrong with the patient’s heart. The NCBI Clinical Methods textbook (Text) states that “The clinical evaluation of the second heart sound has been called the "key to auscultation of the heart." However, the remainder of the assessment of the second sound was not taught ala the Clinical Methods. Relying only on the limited sense of the physiological split of the second sound provided a false sense of security – it means nothing until the second sound has been assessed fully and professionally.
  2. It was not taught at postgraduate internal medicine level in the United Kingdom during the early 1990s when I passed the MRCP (UK) examinations. What the current state of affairs is, I do not know.
  3. It is not taught at postgraduate paediatrics level – at least not in South Africa, as has been seen in the second patient's case above.

On realising my shortcomings, I started to examine and re-examine meticulously all patients predisposed to PAH – e.g. patients from atopic backgrounds, with or without asthma, whooping cough, or bronchitis, as well as patients having suffered some degree of hypoxia during birth. I found almost invariably a persistent split of the pulmonary second sound during expiration, but particularly during an extended expiration, and not during the valsalva maneuver, in the sitting or standing position. The Clinical Methods (Text) says the following:

"Therefore, the presence of audible splitting during expiration (i.e., the ability to hear two distinct sounds during expiration) is of greater significance at the bedside in identifying underlying cardiac pathology than is the absolute inspiratory increase in the A2 --P2 interval."

"In almost all patients with heart disease and audible expiratory splitting in the recumbent position, expiratory splitting persists when the patient is examined in the sitting or standing position. Thus, the finding of audible expiratory splitting in both the recumbent and upright positions is a very sensitive screening test for heart disease."

I recommend my colleagues to study this chapter scrupulously. I will not discuss other disturbances of the second sound.

How common is it and why does this matter?

I confirmed the condition particularly in atopic patients. There are very many patients with this condition, since it is estimated that 20-30% of 48 million South Africans suffer from the allergy problem (PDF). Thus, South Africa may have about 12 million patients suffering from mild PAH. The non-specific nature of the condition generates medically unexplained symptoms (MUS). It is the cause of undiagnosed and misdiagnosed angina pectoris in the young and not so old. It is a rate limiting factor for many other illnesses, especially eczema were the therapies dealing with both eczema and mitral and/or tricuspid regurgitation must be employed. As such, these patients can be at risk of sudden death while in a seemingly healthy state yet having angina symptoms misdiagnosed even by cardiologists.

How is it diagnosed?


  1. Fatigue.
  2. Poor stamina.
  3. Chest pain on exertion, improving during rest - angina pectoris.
  4. Nausea on waking, persisting during the rest of the day, with poor appetite especially in young children and teenagers. These children display disruptive and anti-social behaviour, as well as poor scholastic abilities. Unsurprisingly, these complaints represent MUS to paediatricians, to whom some of my patients have been.
  5. Hypoglycaemia which results in a headache. The headache improves on eating.
  6. Deep venous thrombosis in patients without predisposing factors.
  7. Mesenteric thrombosis which led to the surgical resection of the gangrenous segment of the descending colon in a female patient of mine.

Cardiovascular system:

  1. Blood pressure lying and standing. A postural drop is recorded which may vary from between 10-30 mmHg in almost all patients. A few patients have a postural drop of up to 45 mmHg. Regrettably, standing BP is rarely recorded in patients – this should be done routinely in all patients.
  2. An increased pulse rate on rising from the lying position which is associated with postural hypotension.
  3. Central venous pressure is mostly normal, but can be raised.
  4. More frequently, c waves are diagnosed due to tricuspid regurgitation.
  5. Left sternal heave is elicited particularly in those patients with chest pain on exertion.
  6. Palpable pulmonary sound may be present or not.
  7. Pulmonary second sound in the pulmonary area is louder than the aortic second sound in the aortic area.
  8. A physiological pulmonary split is found significantly even in adults above 40 years of age. Much less frequently is the absence of a split of the second sound.
  9. A persistent split of the pulmonary second sound during expiration. If this sign is not elicited during quiet normal expiration in a patient suspected of suffering from mild PAH where orthostatic hypotension has already been recorded, the sign will then almost always be elicited during an extended expiration in the sitting or standing position. This sign must be sought all over the pulmonary area as it is never only located in the typical pulmonary area. The sign may even be elicited on the sternum opposite to the 2nd or 3rd intercostal cartilages.
  10. A constant variable split of the pulmonary sound which varies in width during normal quiet breathing.
  11. Physiological split of the second sound at the apex – an abnormal finding.
  12. 3rd/ 4th right ventricle sounds. Tricuspid and/or mitral regurgitation murmur.


Lungs are often unremarkable, or secondary conditions may be present.


A tender hepatomegaly may be elicited, but seldom so.
Very commonly ascites (shifting dullness) can be elicited


The physical diagnostic information discussed in the NCBI Clinical Handbook (Text) is very important. Worrying is the fact that two patients’ mild PAH was not confirmed by cardiologists via echocardiography. What is also worrying is the fact that both colleagues did not perform the required auscultatory maneuver. 

The question emanating from the failure of echocardiography to confirm mild PAH is: can this common condition only be diagnosed via invasive catheter studies? If so, auscultatory skills become extremely important as ethics would preclude mass invasive investigations of relatively healthy young patients.

How is it treated?

The main treatment is Cactus Grandiflorus:

  1. Cactus 7x potency: 5 drops of a liquid potency twice daily which I dispense in a 30ml dropper bottle.
  2. Increase potency every 4-5 weeks with a further 2x (7x is increased to a 9x), or depending on symptoms.
  3. Additional treatment: 5 drops of Cactus is administered during and after sporting activities. This is to improve pulmonary circulation, decrease central venous pressure which backs up and induce a sluggish mesenteric venous return due to the much higher cardiac output during exercise. This I deduced to be the cause of the mesenteric thrombosis I alluded to earlier. This phenomenon is also responsible for the fact why some patients of mine experienced an increase in their body weight by up to 3 kg with an increase in their abdominal girth within 12 hours after a gym session. The phenomenon is due to the accumulated ascites.
  4. For patients having angina, Cactus 200c is provided when needed – 5 drops, 3 times, 5 minutes apart. Repeat when needed.

I do not discuss the treatment of all associated conditions which I regularly diagnose with such patients.

The outcomes!

I schedule follow-up of the patients at about 4-5 weeks, monitoring their stamina and chest pains which should improve. Children who had a poor appetite with nausea eat better within a few days, with a disappearance of their nausea and with an associated improvement of their personality. Similarly, children who are very frequently hungry eats less frequently while their behaviour and scholastic abilities improve. All the above symptoms and signs are used to adjust their medication.

I have noted that the low blood pressure, orthostatic hypotension and ascites improve very slowly. As such I record these investigations now about every 4 months. However, I have confirmed the disappearance of the abnormal persistent split of the second sound in expiration, and particularly, during an extended expiration, in a number patients; however, stopping the Cactus too quickly caused a minimal recurrence of it.

© Dr HJD Jeggels 2010.